{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T17:46:05Z","timestamp":1792086365262,"version":"4.4.0"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T00:00:00Z","timestamp":1748476800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T00:00:00Z","timestamp":1748476800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100001774","name":"University of Sydney","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001774","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Graefes Arch Clin Exp Ophthalmol"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Purpose<\/jats:title>\n            <jats:p>The electronegative electroretinogram (ERG) is a specific clinical finding usually indicating inner retinal dysfunction occurring post-phototransduction. X-linked retinoschisis (XLRS) and complete and incomplete congenital stationary night blindness (cCSNB, iCSNB) are inherited retinal dystrophies classically associated with electronegative ERGs. Comparing the full-field ERG b:a ratio expands current ERG diagnostic criteria and aids in localising physiological sites and pathological mechanisms.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>A retrospective review of patients with a clinical diagnosis of iCSNB, cCSNB and XLRS was conducted. ERG and genetic results were analysed. Average b:a ratios between groups were compared, and prevalence of electropositivity was assessed using thresholds of b:a\u2009&gt;\u20091.0 and b:a\u2009&gt;\u20091.50.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>53 patients were included, and genetic confirmation was available in 7\/24 iCSNB, 3\/14 cCSNB and 11\/15 XLRS patients respectively. In genetically proven cases, mean b:a ratio in XLRS patients (b:a\u2009=\u20091.04) was significantly higher than cCSNB (b:a\u2009=\u20090.60, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) and iCSNB (b:a\u2009=\u20090.60, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). An electropositive ERG was significantly more likely to be associated with RS1 than iCSNB (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) or cCSNB (<jats:italic>p<\/jats:italic>\u2009=\u20090.001) at b:a\u2009&gt;\u20091.0 threshold, and more likely RS1 than iCSNB (<jats:italic>p<\/jats:italic>\u2009=\u20090.040) at b:a\u2009&gt;\u20091.5 threshold.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>Our study highlights the distinct ERG findings between these typically electronegative inner retinal dystrophies. In a clinical setting, the traditional electronegative definition of b:a\u2009&lt;\u20091.0 appears very insensitive to detect XLRS patients. Our data suggests clinical suspicion should remain even in patients with a b:a ratio\u2009&gt;\u20091.50, and highlights the importance of genetic testing in these cases.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00417-025-06851-4","type":"journal-article","created":{"date-parts":[[2025,5,28]],"date-time":"2025-05-28T23:37:20Z","timestamp":1748475440000},"page":"2247-2255","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Understanding the phenotype of genetically associated electronegative ERG retinopathies: comparing the full-field ERG b:a ratio"],"prefix":"10.1007","volume":"263","author":[{"given":"Christopher A.","family":"Ovens","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa E.","family":"Cornish","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haipha","family":"Ali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vannessa","family":"Leung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dhimas H.","family":"Sakti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nonna","family":"Saakova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marium","family":"Raza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin M.","family":"Nash","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clare L.","family":"Fraser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"McCluskey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn V.","family":"Jamieson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R.","family":"Grigg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,5,29]]},"reference":[{"key":"6851_CR1","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1111\/ceo.14065","volume":"50","author":"DH Sakti","year":"2022","unstructured":"Sakti DH, Ali H, Korsakova M, Saakova N, Mustafic N, Fraser CL, Jamieson RV, Cornish EE, Grigg JR (2022) Electronegative electroretinogram in the modern multimodal imaging era. 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This study was approved under the low-negligible risk pathway.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"A waiver of consent was granted for this retrospective study. All patients had previously signed an informed release of information statement to facilitate this.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"Authors RV Jamieson and JR Grigg are both supported by Belite Bio. All other authors have no relevant financial or non-financial interests to disclose. All other authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers\u2019 bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}