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Jack Russell progressive retinal atrophy, Jack Russell terrier
progressive retinal atrophy, Conquest Terriers, Brantford, Ontario, Canada.
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Serving Brantford, Hamilton, London, Milton, Toronto, Niagara, St.
Catharines, Fort Erie, Buffalo New York.
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Progressive Retinal Atrophy
Progressive
retinal atrophy, or PRA as it is frequently termed, is a
long recognized, hereditary, blinding disorder. The first
modern description of this problem was in Gordon Setters in
Europe, in the early years of the twentieth century, but
since then PRA has been recognized in most purebred dogs.
PRA is a disease of the retina. This tissue, located inside
the back of the eye, contains specialized cells called
photoreceptors that absorb the light focused on them by the
eye's lens, and converts that light, through a series of
chemical reactions into electrical nerve signals. The nerve
signals from the retina are passed by the optic nerve to the
brain where they are perceived as vision. The retinal
photoreceptors are specialized into rods, for vision in dimlight (night vision), and cones for vision in bright
light (day and color vision). PRA usually affects the rods
initially, and then cones in later stages of the disease. In
human families the diseases equivalent to PRA (in dogs) are
termed retinitis pigmentosa.
In all canine
breeds PRA has certain common features. Early in the
disease, affected dogs are nightblind, lacking the ability
to adjust their vision to dim light; later their daytime
vision also fails. As their vision deteriorates, affected
dogs will adapt to their handicap as long as their
environment remains constant, and they are not faced with
situations requiring excellent vision. At the same time the
pupils of their eyes become increasingly dilated, causing a
noticeable "shine" to their eyes; and the lens of their eyes
may become cloudy, or opaque, resulting in a cataract.
The big
difference in PRA among breeds is in the age of onset and
the rate of progression of the disease. Certain breeds,
notably including the Collie, the Irish Setter, the
Norwegian Elkhound and the Miniature Schnauzer, have early
onset forms. In these breeds the disease results from
abnormal or arrested development of the photoreceptors --
the visual cells in their retina, and affects pups very
early in life. In other breeds, including the Miniature
Poodle, the English and American Cocker Spaniel, and the
Labrador Retriever, PRA is much later in onset. Affected
dogs in these breeds appear normal when young, but develop
PRA as adults.
Diagnosis of
PRA is normally made by ophthalmoscopic examination. This is
undertaken using an instrument called an indirect
ophthalmoscope, and requires dilatation of the dog's pupil
by application of eyedrops. Broadly speaking all forms of
PRA have the same sequence of ophthalmoscopic changes:
increased reflectivity (shininess) of the fundus (the inside
of the back of the eye, overlain by the retina); reduction
in the diameter and branching pattern of the retina's blood
vessels; and shrinking of the optic nerve head (the nerve
connecting the retina to the brain). These changes occur in
all forms of PRA, but at different times in the different
breed-specific forms. Usually by the time the affected dog
has these changes there is already significant evidence of
loss of vision.
Confirmation
of the diagnosis can be undertaken by electroretinography.
This is an electrical measurement of retinal function
somewhat similar to an electrocardiographic test of heart
function, but with two differences: the electroretinogram
(ERG) can only be recorded as a response to a flash of light
(ie: it is not a free running signal like the EKG); and
accurate recording of the ERG requires that the dog be
anesthetized. In all dogs showing clinical evidence of PRA,
the ERG is severely diminished or extinguished.
The ERG can
also be used for early diagnosis of specific forms of PRA,
that is to detect PRA-affected dogs before they demonstrate
clinical evidence of disease. This requires very carefully
controlled ERG recording conditions, and a well defined
understanding of the age of onset and rate of change of ERG
dysfunction in the specific form of PRA under consideration.
Written by
Gregory Acland |
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Conquest Jack Russell Terriers
For Work, Show or Just
Plain Lovin'
RR#2 Brantford Ontario N3T 5L5
519-484-2509 phone
519-484-9918 fax
conquestterriers@gmail.com
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