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In Staffordshire Bull Terriers this is a neurometabolic disorder characterised by elevated levels of L-2-hydroxyglutaric acid in urine, plasma and cerebrospinal fluid.
L-2-HgA affects the central nervous system, with clinical signs usually apparent between 6 months and one year (although they can appear later). Symptoms include epileptic seizures, "wobbly" gait, tremors, muscle stiffness as a result of exercise or excitement and altered behaviour.
The mutation responsible for the disease has recently been identified at the Animal Health Trust. Using the information from this research, we have developed a DNA test for the disease. This test not only diagnoses dogs affected with this disease but can also detect those dogs which are carriers, displaying no symptoms of the disease but able to produce affected pups.
Carriers could not be detected by the tests previously available which involved either a blood or urine test detecting elevated levels of L-2-hydroxyglutarate or magnetic resonance imaging. Under most circumstances, there will be a much greater number of carriers than affected animals in a population. It is important to eliminate such carriers from a breeding population since they represent a hidden reservoir of the disease that can produce affected dogs at any time.
The test is available now and information on submitting samples is given below.
Breeders will be sent results identifying their dog as belonging to one of three categories:
CLEAR: the dog has 2 copies of the normal gene and will neither develop L-2-HgA, nor pass a copy of the L-2-HgA gene to any of its offspring.
CARRIER: the dog has one copy of the normal gene and one copy of the mutant gene that causes L-2-HgA. It will not develop L-2-HgA but will pass on the L-2-HgA gene to 50% (on average) of its offspring.
AFFECTED: the dog has two copies of the L-2-HgA mutation and is affected with L-2-HgA. It will develop L-2-HgA at some stage during its lifetime, assuming it lives to an appropriate age.
Samples for testing (3mls blood in an EDTA tube), should be sent together with a completed DNA Testing form and a cheque for £63-00 (inc VAT) for each sample to Genetic Services, Animal Health Trust, Lanwades Park, Kentford, Newmarket, Suffolk CB8 7UU. If you also have the tests for HC - Heridatary Cataracts done at the same time you will incur a saving. DNA testing forms can be obtained by contacting Vikki Lett 08700 509144 or via e-mail to vikki.lett@aht.org.uk.
HEREDITARY CATARACTS (HC)
Hereditary Cataracts, facts. Hereditary Cataracts are a disease which have blighted the Staffordshire Bull Terrier world for many, many years.
There has been NO way of telling whether your dog was a carrier of this heart breaking gene in the past.
Now, we are in the marvellous position of being able to have our dogs genetically tested for the gene. A simple blood test, sent off to the Animal Health Trust, will tell us uncategorically that our dog is either:
1) clear of the gene (therefore unable to pass it on to progeny).
2) a carrier of the gene (therefore may pass it on to progeny).
3) affected by the disease (therefore has received a copy of the gene from BOTH parents and WILL develop Hereditary Cataracts).
A carrier animal will NEVER develop cataracts themselves. If mated to a non carrier animal, a percentage of the pups could also be carriers and a percentage clear. If mated to another carrier, a percentage will be AFFECTED, a percentage carriers and a percentage clear.
If 2 affected animals were mated together (god forbid!) all the pups would be affected.
POSTERIOR POLAR SUBCAPSULAR CATARACTS - (PPSC)
This is usually common in breeds such as the Labrador but apparantley it is now showing up in SBTs. One vet has seen 8 or 9 affected dogs in the last few months. They *think* it has appeared through a mutated gene but have little info on the mode of inheritance in the SBT or whether it is a recessive or a dominant gene.
SBTs should now be tested ANNUALLY as even if they show clear/unaffected by PHPV or HC they can still be affected by PPSC. It can show clear through testing even up to ages 10 or so and then the dog can show as afffected.
Here is some information on the condition:
1) PPSC is a recognised eye disease in several breeds already.
2) PPSC has an, as yet, unknown mode of inheritance.
3) PPSC has a variable age of onset (from 10 months to 10 years and more have been reported).
4) PPSC is not usually noticed by the owner or their vets as only a BVA eye test can detect it.
5) PPSC usually remains a small cataract but some PPSCs can progress to blinding catatracts in some unfortunate individuals.
6) PPSC cannot be detected by litter screening.
7) PPSC is unrelated to juvenile HC as seen in the stafford.
8) PPSC cannot be detected by DNA testing as yet.
9) PPSC in breeds such as the Labrador has led to the development of breeders demanding a *current* clear eye certificate (within the last 12 months) from stud and bitch owners before mating is allowed.
Dogs can prove unaffected for many years and then test affected- HOWEVER they will have been genetically affected ALL THE TIME ! This makes control difficult.
Investigation into the mode of inheritance is being hampered by only checking SOME dogs OCCASIONALLY, rather than EVERY dog EVERY year!
Annual testing is recommended even after breeding has ceased.
There will be times when a dog passes the annual test, mates and has pups and then at the next annual test is found to be positive. The breeder who has taken steps to annually test cannot be held at fault.
Please be aware and make others aware of this condition we should try to find out how this is passed on and the percetage of SBTs becoming affected.
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