Abstract
Full-field electroretinography (ERG) has long been recognized as a useful tool for the detection and characterization of retinal degenerative disease (RDDs). Traditional ISCEV-standard ERGs separate rod-mediated from cone-mediated responses, but can only discriminate among patients on the basis of peak-to-peak amplitude and b-wave implicit time. In an attempt to broaden the phenotypic assessment to better match genetic heterogeneity, we have sought to identify physiologically relevant parameters in the photoreceptor component that relate to activation and deactivation parameters of phototransduction. These new parameters help discriminate among patients with distinct disease-causing mutations.
Clinical trials in RDDs benefit from the detection of sub-microvolt responses in advanced disease and from the analysis of local responses from discrete regions. To provide a more comprehensive analysis of local function, we are attempting to relate multifocal ERG amplitudes and static perimetric thresholds to fine structural measures obtained through spectral domain optical coherence tomography (SD-OCT).