Progressive Retinal Degeneration in Dogs

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Progressive retinal degeneration or atrophy (PRD/PRA) represents a group of inherited eye diseases characterized by abnormal development (dysplasia) or premature degeneration (deterioration) of the retina. 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.

  • Fundic photograph of a 9-year-old male miniature poodle. Note the hyper-reflectivity of the tapetal retina dorsal to the disc, narrowing of the retinal vessels and pallor to the optic disc. Photo provided courtesy of Dr. Rhea Morgan.

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

    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

    No therapy is available to prevent, slow the progression of, or reverse the degenerative changes of PRD/PRA.

    Early diagnosis of PRD using electroretinography or genetic testing is important in kennels to eliminate individuals from the breeding pool that are either clinically affected or represent genetic carriers of the disease.

    Home Care

    Care consists of providing a consistent and safe environment for pets with vision loss. Considerations include:

  • Establish a known location for the food and water bowls and guide your pet to them until he can memorize the locations.

  • Place barriers across staircases, over hot tubs and around pools.

  • Restrict activity on balconies so that small dogs cannot fall through the space between the guardrails and large dogs cannot unwittingly jump off the balcony.

  • Avoid changing the location of the furniture and leaving chairs or other objects out of place in the house. Your pet will memorize a familiar environment in a relatively short time.

  • Purchase toys that squeak, balls that contain bells, or other noisemakers to encourage and help blind dogs to play.

  • Pets affected with PRD lose their vision in dim lighting first. Therefore, night-lights or plug-in hall lights are helpful for negotiating in the house at night.

    Preventative Care

    No preventive care is available for an individual because PRA is genetic. Do not breed affected animals.

    Genetic testing is available for about 15 breeds of dogs affected with PRA. Testing performed on a blood sample can identify which dogs are affected and which are carriers of the disease. This information can then be used by breeders to decide which dogs may or may not be used for breeding. In addition, dogs can be examined on a yearly basis by a veterinary ophthalmologist and certified to be clinically free of the disease. The certification is valid for a period of one year from the time of examination.

  • Veterinary care often includes diagnostic tests to confirm the present of PRD and to exclude other diseases. Tests may include:

  • A complete medical and ophthalmic history. Information of particular importance includes any known information regarding retinal diseases in related animals; the duration and pattern of onset (sudden or slowly progressive) of vision loss; any physical abnormalities accompanying the vision loss; and any medications currently given to the pet. A thorough physical examination is performed to determine whether abnormalities are confined only to the eye or involve other organs in the body.

  • 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 navigate 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 the tick borne diseases (Lyme disease, ehrlichiosis, etc.), fungal diseases and toxoplasmosis
    – A measurement of systemic arterial blood pressure to rule out high blood pressure
    – Possibly immune tests
    – Possibly chest and abdominal X-rays
    – Ultrasonography of the structures behind the eye
    – CT scan or MRI of the brain and vision pathways leading to the brain
    – Cerebral spinal fluid tap to evaluate the fluid around the brain

  • An electroretinogram may be indicated if the retina appears to be the source of the blindness based upon the physical and eye examination findings. This test is essential for confirming the diagnosis of PRD, and to rule out SARD and hemeralopia.

  • If the dog is purebred and belongs to one of the breeds for which genetic testing has been developed, the PRA can be confirmed with a blood test. Genetic testing is performed by Optigen, LLC of Ithaca. NY.

  • There is no treatment for PRD. Most forms of the disease are inherited as a simple autosomal recessive trait, which allows many dogs to be silent carriers of the disease. This inheritance pattern, combined with the fact that many dogs do not show signs until after they are older, make the disease very difficult to eradicate in some breeds of dogs.

    For the 15 breeds of dogs in which genetic testing has been developed (www.optigen.com), blood testing of potential breeding dogs is the best way to identify both carriers and dogs that will show clinical signs. For all other breeds, electroretinography can be performed as a screening tool to detect the disease prior to the onset of clinical signs and retinal abnormalities.

    The most common screening test used for PRD/PRA in breeding dogs is the annual examination of their eyes by a veterinary ophthalmologist. Results of this exam are then forwarded to the Canine Eye Registry Foundation (CERF, www.vmdb.org), an organization that keeps data on the eyes of many purebred dogs in the United States. If the dog is found to be free of inherited eye disease, then it is issued a clearance number by CERF that is good for one year

    As more CERF examinations are performed and as more genetic tests are developed, hopefully breeders will be able to avoid using affected dogs and carrier dogs in their breeding programs and the incidence of PRA will decrease with time.

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