Perceptual Visual Acuity Declines With Age in a Rat Model of Retinitis Pigmentosa While Light Perception is Maintained.

PURPOSE: Retinitis pigmentosa (RP) is a leading cause of blindness and genetically induces impairment of the retinal epithelium and photoreceptors. In this study, we investigated the decline in the visual response and visual ability during disease progression. This understanding is crucial for disease staging in patients, establishing therapeutic plans in advance, and evaluating the effects of interventional treatments.

METHODS

We used a rat model of inherited RP (Royal College of Surgeons [RCS] rats) and evaluated form visual acuity and light perception using behavioral tests and electrophysiological recordings in the dorsal lateral geniculate nucleus, superior colliculus, and primary visual cortex.

RESULTS

The perceptual form vision (detection of grating stimulus) was attenuated by 9 weeks old. The neural responses in the three early visual areas to flashing grating stimuli with various contrasts and spatial frequencies showed similar degeneration progress as the behavioral evaluations. Light perception (detection of a bright uniform light source) was maintained until at least 11 weeks old. The neural responses to the uniform flashlight stimulus in the three early visual areas were maintained during the same period.

CONCLUSIONS

Our findings suggest that form vision is primarily affected by the progression of RP, whereas non-form vision is potentially robust to retinal degeneration. This maintenance of light perception is likely due to the preserved function of intrinsically photosensitive retinal ganglion cells. These results provide useful and fundamental knowledge for evaluating the protective or restorative effects of experimental treatments for RP.