Electrophysiology in some animal and human hereditary diseases involving the retinal pigment epithelium

Abstract

The present paper surveys slow electrophysiological responses recorded by a d.c. technique in some hereditary eye diseases involving the retinal pigment epithelium (RPE) in animals (English setter dogs and Polish Owczarec Nizinny (PON) dogs with ceroid lipofuscinosis and Briard dogs with a slowly progressive rod-cone dystrophy associated with RPE inclusions) and in humans (Best's disease). The electroretinogram c-wave was typically either decreased in amplitude, lacking or replaced by a negative wave. These c-wave changes could be seen at fairly early stages of disease, when the a- and b-waves of the electroretinogram were still within normal limits.