Electroretinograms in English setters with neuronal ceroid lipofuscinosis

Full-field electroretinograms (ERGs) were recorded from three adult English setters with advanced neuronal ceroid lipofuscinosis and three normal controls. Affected setters showed 30% to 40% reductions in a-wave and b-wave amplitudes, normal cone and rod b-wave implicit times, and slightly elevated a-wave and b-wave thresholds. The ERGs of these affected setters differed from those that have been recorded from humans with neuromal ceroid lipofuscinosis (Batten's disease), humans with hereditary retinitis pigmentosa, Irish setters with rod-cone dysplasia, and miniature French poodles with progressive rod-cone degeneration.