Studies on the retina and the pigment epithelium in hereditary canine ceroid lipofuscinosis. IV. Changes in the electroretinogram and the standing potential of the eye.

The present study correlates ultrastructural abnormalities in the retinal pigment epithelium (RPE) with electrophysiologic changes demonstrated in examinations of dogs with canine ceroid-lipofuscinosis (CCL) at various stages of clinical involvement using dc electroretinograms (ERG), including the c-wave, as well as recordings of the variations of the standing potential (SP) of the eye, both of which reflect the activity of the pigment epithelium. Normal c-waves and SP variations were seen at early stages of disease. At later stages, specific signs of pigment epithelial impairment were found in the form of (1) markedly reduced SP variations, (2) an increase in amplitude of the trough after the b-wave, and (3) a disappearance of the c-wave, which was replaced by a negative potential. These ERG changes correlate very well with a damaged pigment epithelium that generates the positive potential of the c-wave, as well as with normal Müller cells that generates the negative potential coinciding in time with the c-wave, and contributing to the trough after the b-wave. In the end-stage of the disease all potentials of the ERG were reduced drastically, evidently as a sign of cessation of neuroretinal function. Similarities between English setters with CCL and human Batten disease are discussed.