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.
D000293:Adolescent; D000328:Adult; D000613:Aminobutyrates / Q000494:pharmacology; D000818:Animals; D004596:Electroretinography / Q000379:methods; D005260:Female; D006801:Humans; D008253:Macaca mulatta; D008266:Macula Lutea / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009755:Night Blindness / Q000151:congenital / Q000503:physiopathology*; D012160:Retina / Q000187:drug effects / Q000502:physiology; D017949:Retinal Cone Photoreceptor Cells / Q000503:physiopathology*
D000613:Aminobutyrates; C012729:2-amino-4-phosphonobutyric acid