Abstract
Background Electroretinogram (ERG) parameters quantify severity in Stargardt disease giving prognostic information. Standard tests take up to an hour. A portable device can record light-adapted ERGs within minutes. We explored whether ERG parameters obtained with the device associated with severity of genotype.
Methods Patients with genetically confirmed ABCA4-retinopathy were recruited to undergo recordings with the device (RETeval system). Variants were classified by severity (mild; mild/moderate; moderate; severe) according to a recent paper. Each patient was classified by severity of the milder allele. ERG b-wave and flicker peak times and amplitudes were compared between the 4 groups.
Results 55 patients were included (mean (SD) age, 51 (16) yrs). Of these, 30, 9, 8 and 8 were classified genotypically as mild, mild/moderate, moderate, severe and respectively. Mean b-wave and flicker amplitudes were progressively smaller as severity increased and peak times were more delayed. Differences between mild and severe groups were significant.
Conclusion Portable ERG parameters correlated with genotype, supporting the reliability of the test in yielding prognostic information.
