ELECTROPHYSIOLOGY IN SOME ANIMAL AND HUMAN HEREDITARY-DISEASES INVOLVING THE RETINAL-PIGMENT EPITHELIUM

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