Comparison of focal macular cone ERGs in complete-type congenital stationary night blindness and APB-treated monkeys

Focal macular cone electroretinograms (ERGs) and multifocal ERGs were recorded to study the macular function in patients with the complete-type of congenital stationary night blindness (cCSNB). The waveforms of the focal macular cone ERGs and the on- and off-responses of the multifocal ERGs in the cCSNB patients were similar to those recorded from monkey retinas treated with L-2 amino-4-phosphonobutyric acid (APB), suggesting that patients with cCSNB have a complete defect of the on-pathway even in the central retina. The results also demonstrated that there was a paradoxical positive response in the central retina of cCSNB patients, as compared to the negative full-field ERGs in the same subjects.