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American Eskimo Health Issues |
Progressive Retinal Atrophy (PRA)
http://www.qualitydogs.com/dog-articles/Dog-Health/progressive-retinal-atrophy.html
Progressive
Retinal Atrophy is a group of eye diseases of the retina, which
lead to blindness. It has actually become a bit of a catch-all
term for a variety of conditions, each with different specific
mechanisms, affecting different breeds at different ages, but
the common factor is the blindness that ultimately results from
the diseased retina.
A brief discussion of the function of the retina will help in
understanding PRA and its various forms. The retina is the
innermost layer of the back of the eye, and it contains the
cells that are sensitive to light (“photoreceptors”) and the
nerves structures that run from those cells through the optic
nerve and, ultimately, to the brain. There are two types of
photoreceptors: rods, which function in dim light, detect shape
and motion, do not differentiate color, and are more
concentrated around the perimeter of the retina; and cones,
which function in brighter light, detect color, and are more
concentrated in the central area of the retina.
The two major categories of retinal diseases typically grouped
under Progressive Retinal Atrophy are (1) retinal dysplasia,
where the key cells of the retina do not develop properly in the
first eight weeks of life, and (2) retinal
degeneration, where the cells do develop normally in the fetus
and early puppyhood, but degenerate later in life. Additionally,
some forms of PRA may affect only the rod cells or only the cone
cells of the retina.
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Symptoms of PRA Although the types and expressions of the forms of Progressive
Retinal Atrophy do vary in severity and age of onset, the common
symptoms are progressive vision loss, almost always beginning
with a loss of night vision, as the disease affects the rods
first. As night vision deteriorates, the dog will typically
exhibit behavioral signs, such as disorientation at night,
apprehension around stairs or new environments in dim light, or
literally getting lost in their own home, especially if the
furniture has been rearranged. Physiologically, affected dogs
will typically have excessively dilated pupils at night, as
their eyes are trying to let in more light. Additionally, there
may be a noticeably greater shine to the eyes at night, as the
pupils dilate excessively and the retinal tissue thins, exposing
more of the reflective rear surface of the eye.
Diagnosis of
Progressive Retinal Atrophy Preliminary diagnosis is typically via fundoscopic exam (with an
ophthalmoscope) by a licensed veterinary ophthalmologist. This exam is
painless and requires no sedation, but the pupil must be dilated. The
vet would be looking for blood vessel shrinkage, increased reflectivity
of the tissue behind the retina, decreased pigmentation of the retina,
and a darkened optic disc in advanced stages. However, secondary
cataract formation, which may occur with the disease, may make
examination difficult. Additionally, depending on the breed and type of
Progressive Retinal Atrophy, these symptomatic changes may not occur
until later in life when the disease begins to manifest, so a clear exam
in a puppy does not guarantee they are free from the disease.
Often, though, a dog will already have exhibited signs of the disease,
like night vision loss, by the time a fundoscopic exam would clearly
identify PRA.
However, a more accurate assessment can be made via an electroretinogram
(ERG), which is an electrical evaluation of the response of the retina
to varying flashes of light and color. This procedure does require
sedation, as it involves placing a type of contact lens over the eye.
This procedure is sensitive and accurate enough to detect changes in the
retina, often before the vision has deteriorated to the point that the
dog shows signs of Progressive Retinal Atrophy.
Treatment of Progressive Retinal Atrophy There is no treatment for PRA, and no known way to slow the progression.
Generally, it eventually progresses to blindness. There have been some
studies on the use of Vitamin A therapy on the human parallel disease,
retinitis pigmentosa, but no studies have been done to date on vitamin
therapy in dogs.
Breeds Affected by PRA Following are some of the breeds affected, type of PRA common within the
breed, age of onset, genetic mode of inheritance, and presence of a
genetic marker test for the breed.
|
Breed |
Type of
PRA |
Age of
Onset |
Inheritance |
Genetic
Test? |
|
Akita |
rod-cone degeneration |
1-3 yrs |
autosomal recessive |
No |
|
Bullmastiff |
Rhodopsin deficiency |
variable |
autosomal dominant |
Yes |
|
Cairn
Terrier |
rod-cone dysplasia |
< 1 yr |
autosomal recessive |
No |
|
Cocker
Spaniel |
rod-cone degeneration |
3-6 yrs |
autosomal recessive |
Yes |
|
Collie |
rod-cone dysplasia |
< 1 yr |
autosomal recessive |
No |
|
Irish
Setter |
rod-cone dysplasia |
< 1 yr |
autosomal recessive |
Yes |
|
Labrador Retriever |
rod-cone degeneration |
4-6 yrs |
autosomal recessive |
Yes |
|
Min. LH
Dachshund |
generalized PRA |
6 mos |
autosomal recessive |
No |
|
Miniature Poodle |
rod-code degeneration |
3-6 yrs |
autosomal recessive |
Yes |
|
Miniature Schnauzer |
rod-code degeneration |
3-6 yrs |
autosomal recessive |
Yes |
|
Norwegian Elkhound |
early
retinal degeneration |
6 wks –
1 yr |
autosomal recessive |
Yes |
|
Papillon |
generalized PRA |
variable |
autosomal recessive |
No |
|
Samoyed |
X-Linked PRA |
3-5 yrs |
X-Linked |
Yes |
|
Siberian Husky |
X-Linked PRA |
2-4 yrs |
X-linked |
Yes |
|
Tibetan
Spaniel |
generalized PRA |
3-5 yrs |
autosomal recessive |
No |
|
Tibetan
Terrier |
generalized PRA |
6-12
mos |
autosomal recessive |
No |
Breeding
Decisions Dogs affected with PRA should obviously not be allowed to breed, and
should be neutered or spayed. Since Progressive Retinal Atrophy is
inherited genetically, parents and siblings of PRA-affected dogs should
also not be bred, in the absence of complete genetic evaluation.
Fortunately, with precise genetic tests now available for many breeds,
affected and carrier dogs can be pinpointed prior to breeding decisions.
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