ArticleGraefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie2025
Understanding the phenotype of genetically associated electronegative ERG retinopathies: comparing the full-field ERG b:a ratio.
Article in Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper, 1 of them a synthesis that pooled it.
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Who cites it
1 citing paper in PubMed, 1 synthesis or guideline pooled it.
- Electronegative electroretinography in inherited retinal disease: a systematic review of genotype-phenotype correlations.BMC ophthalmology · 2026Pooled it
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12 authors.
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No grant is acknowledged in the PubMed record.
Abstract
purposeThe 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.
methodsA 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 > 1.0 and b:a > 1.50.
results53 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 = 1.04) was significantly higher than cCSNB (b:a = 0.60, p < 0.001) and iCSNB (b:a = 0.60, p < 0.001). An electropositive ERG was significantly more likely to be associated with RS1 than iCSNB (p < 0.001) or cCSNB (p = 0.001) at b:a > 1.0 threshold, and more likely RS1 than iCSNB (p = 0.040) at b:a > 1.5 threshold.
conclusionOur study highlights the distinct ERG findings between these typically electronegative inner retinal dystrophies. In a clinical setting, the traditional electronegative definition of b:a < 1.0 appears very insensitive to detect XLRS patients. Our data suggests clinical suspicion should remain even in patients with a b:a ratio > 1.50, and highlights the importance of genetic testing in these cases.
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