Progressive Retinal Atrophy 5, PRA5
Progressive retinal atrophy (PRA) describes a group of non-painful inherited disorders of the retina's photoreceptor cells that result in vision loss in dogs. PRA caused by this NECAP1 variant is a late-onset (later in life) form of PRA, affecting dogs whose eyes have completed their normal development.
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Signs and symptoms
A dog with decreased vision may be reluctant to walk down stairs, enter a dark room, or go outside at night. They may bump into door frames or corners and have difficulty fetching toys. You may notice a characteristic eyeshine due to increased reflectivity of the tapetum, the layer of the eye that reflects light. The dog's discernible visual impairment will typically lag behind changes observed by a veterinarian and electroretinogram (ERG) abnormalities.
This form of PRA is typically diagnosed at around four years old. -
Diagnosis
PRA is diagnosed by examination of the back of the eye (the fundus). In the early stages, observing changes to the retina may be difficult. Still, as the disease progresses, a veterinarian will notice increased reflectivity and thinning of the retinal blood vessels. Changes affect both eyes equally, helping to distinguish PRA from other retinal diseases. A veterinary ophthalmologist can perform an ERG, which measures the electrical activity, and thus the function, of the retinas.
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Treatment
Currently, there is no widespread treatment for progressive retinal atrophy. However, gene therapy is an evolving field.
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What to do if your dog is at risk
Actions
- Because the condition is progressive, dogs will adapt to the gradual vision loss over time. Owners should help affected dogs navigate their homes and the outside world by keeping furniture in the same location, ensuring they are on a leash in unfamiliar territory, and training them to understand verbal commands or using scent markers.
- A dog may develop cataracts later in the disease progression. Oral antioxidant therapy has been shown to improve retinal function in normal dogs and decrease oxidative stress on lens cells, which can help delay cataract formation. Ask your vet about supplements to give to your dog.
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Genetic Information
This variant was first described in the Giant Schnauzer.
This variant is inherited in an autosomal recessive manner, meaning a dog requires two copies of the variant to show signs of PRA5.
Gene names:
NECAP1 Exon 6 ‐ chr
Inheritance type:
recessive
Citations:
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Breeds affected
This health condition affects the following breeds
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