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- Title
Effects of hereditary retinal degeneration due to a CEP290 mutation on the feline pupillary light reflex.
- Authors
Thompson, Stewart; Whiting, Rebecca E. H.; Kardon, Randy H.; Stone, Edwin M.; Narfström, Kristina
- Abstract
Objective To understand how progressive rod cone degeneration due to a mutation in CEP290 affects the pupillary light reflex (PLR) in domestic cats. Animals studied Domestic cats identified as either normal wildtype (WT; n = 6), or homozygous for the rdAc mutation in CEP290 and having early stage retinal degeneration (stage 2, S2; n = 4), or advanced retinal degeneration (S4; n = 6). Methods The effect of light on pupil size was measured over a series of 10-s pulses of white and chromatic light in cats lightly sedated with medetomidine. Results In WT cats, the PLR was characterized by a pronounced initial constriction that rapidly re-dilated during the stimulus (pupil escape), to a stable or sustained constriction. There was then a marked constriction at stimulus offset. Each component of the PLR was retained in affected cats, but with progressively reduced irradiance sensitivity from early to advanced retinal disease. Conclusions The PLR of cats had multiple phases, with a remarkably high-amplitude ‘paradoxical’ off-constriction even in the absence of retinal disease. In rdAc cats, reduced irradiance sensitivity was consistent with progressive loss of rod and cone function. Based on previously characterized retinal pathology, this suggests the visual streak of the retina has a proportionally large contribution to PLR input. These findings support the hypothesis that the efficacy of planned therapeutic trials can be determined by careful evaluation of the PLR in cats.
- Subjects
RETINAL degeneration; RETINAL diseases; CAT diseases; PUPIL (Eye); PATHOLOGY
- Publication
Veterinary Ophthalmology, 2010, Vol 13, Issue 3, p151
- ISSN
1463-5216
- Publication type
Article
- DOI
10.1111/j.1463-5224.2010.00772.x