Progressive Retinal Atrophy
Progressive Retinal Atrophy (PRA) is a genetic disease resulting in the progressive loss of function of the rods and cones in the retina (also called Progressive Rod Cone Dysplasia or prcd). In poodles, this seems to occur after 1 year of age (in some dogs it can happen as a pup) and is known to always be bilateral and resulting in blindness. This is not an immediate loss of vision, and because dogs adjust so well through heightening of other senses and a familiarity with their typical surroundings, the disease may not be appreciated by the family until quite progressed, making one think the course was rapid.
The first symptom is often a loss of night vision (nyctalopia) or a difficulty in transitioning from a lighted area to a dark area. Maybe a failure to be able to focus on a dropped cotton ball in a dark room might be an indication that early degeneration is occuring. The destruction of rods, which are responsible for low light vision accounts for this first symptom. A retinal exam will progressively be able to find areas of retina that are abnormal (hyperreflectivity) and an Electro RetinoGram (ERG) can often pick up problems very early.
Hyperreflectivity of the tapetum (the light reflecting portion of the retina) and attenuation or decreased size of the blood vessels in an animal with prcd-PRA
With time, vision will decrease to complete blindness. This occurs as the cones, which are responsible for higher light level vision (responsible for color vision in people) are destroyed. The pupil diameter in normal lighting will be enlarged compared to a normal dog as the retina becomes desperate to attract as much light as possible. This seems to be an autosomal recessive genetic disorder in most cases and is not treatable. Patients do not experience pain and often have no other ocular abnormalities (although poodles are also prone to cataracts which are a separate problem).
The classical screening technique has been through CERF exams (Canine Eye Registration Foundation). A board certified veterinarian will do a thorough retinal exam and look for lesions suggestive of prcd-PRA. The problem is that the time of onset in affected animals may vary, and a single CERF exam does not rule out the possibility that an animal has the problem, and especially will not pick up carriers. Consistent annual CERFing of breeders and responding appropriately to reports of affected offspring is necessary to prevent this problem for progressing and needlessly ruining dog's lives. Genetic testing is currently available for miniature and toy poodles to identify prcd-PRA. At this time, OPTIGEN does not test standards, but hopefully this will become available with time? They currently have not received genetic profiles or CERF reports on Standards suggesting that this is a primary problem. "The cases of affecteds are apparently rare, and we have not identified sufficient affecteds, or pedigrees with affecteds, to determine if they inherit the prcd form of PRA,or another form. If you see a PRA affected standard, and the owner is willing to provide copies of the eye exam report, pedigree and a bloodsample, we'd be very interested in obtaining these." ---Jeanette S. Felix, Ph.D., President and Manager,OptiGen, LLC. Even if Standard Poodles are found to have PRA in sufficient numbers to result in the identification of a genetic marker, this will not likel displace CERF testing. In toy and miniature poodles where PRA is a big problem, only 75% of poodle degenerative retinal disorders are from the genetically identified problem. There are other PRA like disorders that account for the remaining retinal degenerations and won't likely be identified by the genetic testing. OPTIGEN would love information on Standards to help them provide service if possible, and asks for a copy of the CERF report diagnosing the problem, the animal's pedigree, and a blood sample so that genetic markers can be identified. If you have knowledge of this problem in Standards... please step out and help Optigen with their ongoing genetic research!