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Seven eyes from 2 generations of Briard dogs (5 weeks — 7 years old) with congenital night blindness and (in the second generation) impairment of day vision to varying degrees, were examined by light and electron microscopy. Specimens from 4 locations were studied: the central area, the midperiphery of the tapetal area, the upper periphery and the lower periphery. Disorientation of rod outer segment disc membranes was seen in the 5-week-old dog. Large electron-lucent inclusions were found in the RPE at 3.5 months of age. These inclusions occurred most frequently in the central and midperipheral-tapetal areas and seemed to increase in numbers and spread towards the periphery with increasing age. The content of these inclusions is not elucidated. Rod photoreceptor degeneration was apparent from 7 months of age and was most prominent in the peripheral areas. The cones were better preserved. The 7-year-old dog showed reduction of photoreceptors in the central and midperipheral-tapetal areas and almost complete photoreceptor degeneration in the periphery. This dog also showed severe changes of the inner retina in the peripheral fundus. It appears that these Briard dogs suffer from a very slowly progressive retinal degeneration, in which the photoreceptor degenerative changes do not correlate anatomically to the changes in the RPE cells. The disease seems to be different from the retinopathy described in the English Briards. It is not clear yet whether the lipid type of retinopathy found in American Briards is identical to the present disease.

Ophthalmoscopic and electroretinographic (ERG) findings were correlated in a group of Abyssinian cats affected by a slowly progressive and hereditary retinal degenerative disease. According to ophthalmoscopic findings the disease was divided into stages. At stage 1 and 2 retinal changes were minor; showing a gray discoloration most often in the peripheral and midperipheral tapetal fundus. At stage 3 discoloration was generalized and there was marked vascular attenuation. A generalized retinal atrophy was found at stage 4. ERG recordings showed an abnormally depressed stimulus response curve for the b-wave at stage 1 of disease when 30-Hz cone flicker responses were indistinguishable from normal. With progression of disease there was a successive decrease of a- and b-wave amplitudes before there was a significant reduction also of the c-wave amplitude (first seen at stage 3). The ERG was nonrecordable at stage 4. These findings suggest that the photoreceptors are affected primarily by the disease, before there is a functional involvement also of the pigment epithelium. The rod system seems to be affected early in the disease as compared with the cone system. A staging of the disease by ophthalmoscopy correlated more to the function of the rods than to that of the cones.

Visual dysfunction and neurological symptoms were found in Polish Owczarek Nizinny (PON) dogs. Two dogs were examined, one at 2 years of age and the other one at 4 years. The oldest dog was totally blind. The 2-year-old dog developed mental disturbances and the 4-year-old dog became severely ataxic. Ophthalmoscopical findings were retinal hyper-reflectivity, attenuation of the retinal vessels and the presence of greyish to brown spots in the fundus. Electrophysiological and ultrastructural studies were performed in the 2-year-old dog. Scotopic ERG responses were absent, whereas 30 Hz cone flicker responses were recordable, although with an amplitude reduced to about 30% of the normal level. A slow negative potential replaced the c-wave, indicating a dysfunction of the RPE. Intracellular inclusions with a granular appearance or containing membranous fingerprint-like or curvilinear profiles, resembling ceroid, were found in different retinal cells. The RPE cells in the central areas were charged with autofluorescent material having similar structure, Photoreceptor degeneration was most severe in the central areas, corresponding to the RPE changes. It appears than the PON dog may provide a new animal model for neuronal ceroid lipofuscinosis.

We used a simple and reproducible technique with full-field electroretinography and a special-purpose computer system to test and evaluate outer retinal function in dogs. The standardized protocol included the following five basic responses: (1) a stable initial light-adapted, mainly cone derived response, (2) a dark-adapted rod response, (3) chromatically separated rod and cone responses, (4) a maximal rod and cone response and (5) an isolated cone flicker response. For evaluating the electroretinographic responses, a graphic-presentation was used that included data from the tested animal as well as normative data from dogs of the specific breed and age group, presented as the percentage of the median in which limits of normality were depicted in percentiles.

Electroretinography was performed in 10 Abyssinian cats, homozygous for a hereditary retinal degenerative disease but still with an ophthalmoscopically normal retina, and in 11 mixed-breed controls, all between the ages of 8 and 104 weeks. A significant reduction of maximum dark-adapted b-wave amplitude was found in affected kittens as young as 8–16 weeks when compared with controls, although there was no major difference in dark-adapted b-wave threshold or implicit time for the b-wave between affected and controls. For cats 33–104 weeks, similar results were obtained except for the b-wave threshold, which was elevated 2.5 log units in one of the affected cats. No significant difference in 30-Hz cone flicker responses were found between affected and controls at any age studied. In the time period 17–32 weeks affected Abyssinian kittens could not be differentiated from controls by means of the electroretinogram. The significant reduction in scotopic b-wave maximum amplitudes in young affected kittens (8–16 weeks) in conjunction with normal thresholds suggests an early drop-out of rods. It is clear that affected kittens can be differentiated electrophysiologically from controls long before there are ophthalmoscopic signs of retinal disease.