Disorder - Retinitis pigmentosa, peripherin-related

Organ Systems Involved
Nervous/sensory

Alternative disorders described within LIDA

Cataracts; Central progressive retinal atrophy (CPRA); Glaucoma; Progressive rod-cone degeneration (PRCD)

Alternative Names
Autosomal recessive retinitis pigmentosa (ARRP); Late onset retinopathy / Late onset retinal degeneration; Inherited rod-cone degeneration; Retinal pigment epithelial dystrophy (RPED); Central progressive retinal atrophy (CPRA)

Presenting Signs

The retina is the light sensitive part of the inner surface of the eye, located at the back of the eyeball. It contains receptors that receive the light as it enters the eye and transform it into image-forming signals that are transmitted to the brain. Retinitis pigmentosa is a progressive disease in which the pigmented cells lining the retina deteriorate. The deterioration is related to peripherin, a protein found on the light receptor cells. Genetic changes in this protein lead to degeneration of the light receptors, and this leads to visual loss and eventual blindness.

Animals with retinitis pigmentosa will show signs of night blindness and loss of peripheral vision (or tunnel vision) at a young age. This is because the rods, the nerve receptors on the retina that receive black and white images, deteriorate. This then progresses to deterioration of the cones, the nerve receptors that receive coloured images at two to three years old. This results in eventual blindness in cats at three to five years of age. An ophthalmologist may be able to see mottled pigmentation on the retina and thinning of the retina in older cats, but no changes until the age of 1.5 to two years. The pigmented spots eventually join together and fade as the retina deteriorates further.

Cats at Risk

Cats aged up to five years

Breeds Affected
Abyssinian
Ocicat
Somali


Treatment

Experimental surgical therapies only (retinal transplantation and retinal microchip implantation). Progression may be slowed using daily Vitamin A supplementation.

PubMed References
Retinitis pigmentosa, peripherin-related

Contributor
Jen Shepherd