Progressive Retinal Degeneration (PRA) in Dogs

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Overview of Canine Progressive Retinal Degeneration 

Progressive retinal degeneration or atrophy, commonly abbreviated and referred to as  PRD or PRA, represents a group of inherited eye diseases characterized by abnormal development (dysplasia) or premature degeneration (deterioration) of the retina in a dog. In the United Kingdom, a type of retinal degeneration is occasionally seen that affects the retinal layer behind the photoreceptors, and this disease is called central PRA. However, in the United States, PRA is primarily a disease of the light detecting cells of the retina (photoreceptors) and is called generalized PRA.

There are two types of photoreceptors in the retina and these are the light-sensitive rods and cones. They are responsible for detecting light and converting it into an electrical signal that travels to the brain. When the photoreceptor cells deteriorate, vision is lost because the animal has no way to generate an image from the light reaching the retina.

The degenerative form of PRA/PRD in dogs initially affects the rods. The rods are responsible for dim light vision; therefore, the dog loses its nighttime vision first. The disorder is progressive and eventually the cones are affected. Over time, the dog slowly goes completely blind. The disease affects both eyes at the same time.

In the dysplastic forms of the disease the rods of both eyes never develop properly, so the dog is born with poor dim light vision, and the cones rapidly degenerate as the puppy ages. The onset of blindness is much more rapid than with the degenerative forms, and the puppies are usually blind before one year of age.

Over 30 different breeds of dogs are known to have inherited forms of PRA, including the Akita, American cocker spaniel, collie, English cocker spaniel, Irish setter, Labrador retriever, miniature dachshund, miniature schnauzer, all three types of poodle, Norwegian elkhound, papillion, Portuguese water dog and Tibetan terrier. The age of onset varies among the breeds. The onset of clinical signs can be as young as six weeks in dogs with the dysplastic form and as late as six to eight years in dogs with the degenerative form. Most forms of PRD/PRA are inherited as a recessive gene, although a sex-linked form occurs in the Samoyed and Siberian husky, and a dominant form is found in the Mastiff. The disease is uncommon in mixed-breed dogs.

Many dogs are not seen until the late stages of disease and have advanced changes in their retinas because they compensate very well as their vision slowly deteriorates. Sometimes the blindness can appear to be sudden in onset, even though it has been developing for months, because the dog may show almost no clinical signs until the last bit of vision has been lost.

What to Watch For

  • Dilated pupils
  • Bumping into objects, reluctance to go out at night, reluctance to go down stairs in dim light, or other signs of blindness
  • Poor vision in dim light or darkness
  • More readily visible eye shine from the back of the eye due to dilation of the pupils
  • Diagnosis of Progressive Retinal Degeneration in Dogs

    Diagnostic tests are needed to recognize PRD/PRA and exclude other diseases. Your veterinarian will probably take a complete medical history and perform a thorough physical examination.

    A complete ophthalmic examination is indicated and involves all of the following tests. Your veterinarian may refer your dog to a veterinary ophthalmologist for completion of some of these tests:

  • Tests to evaluate vision, such as observing the dog as he navigates an obstacle course in both bright and dim light, and certain neurologic reflex testing
  • Pupillary light reflex testing
  • A Schirmer tear test and fluorescein staining of the cornea
  • Tonometry to measure the pressure within the eye
  • Specialized examination of the front chamber of the eye, the iris and lens, the vitreous and the retina.

    If your veterinarian is concerned that some disease other than PRA is the source of the dog’s blindness, then medical tests to rule out other causes may include the following:

  • A complete blood count (CBC) and serum blood tests
  • Blood tests for some of the tick borne diseases, fungal diseases and toxoplasmosis to look for the presence of infectious diseases
  • A measurement of systemic arterial blood pressure to rule out high blood pressure
  • Possibly chest and abdominal X-rays

    PRA can sometimes be confirmed at the time of retinal examination because it causes characteristic changes in the appearance of the retina. Early stages of the disease can be more difficult to diagnose, and in that instance the disease can be detected with the following test:

  • An electroretinogram to evaluate the function of the photoreceptor cells when they are stimulated with flashes of light. If the electroretinogram is abnormal, then the retina is diseased. If the electroretinogram is normal, then the origin of blindness is somewhere other than the retina.

    Treatment for Dogs with Progressive Retinal Degeneration 

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