L2 - HGA in a mixed breed?

Hello All,

I have a question about L2 HGA. I have recently found out that my dog is mixed with a Staffordshire Bull Terrier. Since he was young he has suffered from seizures, he is wobbly and has tremors. He is on medication for epilepsy, but he does not respond to it the same as most epileptic dogs (according to my vet) but their is no tumor or anything that can be found.

Since learning (only yesterday) that he is mixed with a Stafford, I have done research on the breed and found L2 HGA. I don't know anything about L2 HGA, there is not a lot that I can find.

My question is, if only one parent is a Stafford, is it possible for the puppies to have L2 HGA? Or do both parents have to be Staffords?

Thank you, and sorry if this is a dumb question!

Don't be sorry. As far as I know, 2 copies of the defective gene, one inherited from each parent have to be present for a dog to be affected by the disease. dogs with one copy of the defective gene and one copy of the normal gene are called "carriers".

I've no experience with it unfortunately but I'm sure another member will pop along shortly, don't be sorry ask away perhaps you could come and introduce yourself
https://staffy-bull-terrier.niceboard.com/f7-new-member-introductions

And of course we'd love to see some pics.

https://staffy-bull-terrier.niceboard.com/t12017-how-to-post-a-photos-onto-the-forum

It is possible yes.

Hi, as mattylee said both parents have to be carriers for pups to be affected. My staff has recently been diagnosed with L2HGA. It's also been found in other breeds as well as staffys. The test is a swab test which costs £48.

What other breeds has it been found in?

its been found in west highland whites and Yorkshire terriers.

L-2-HGA (L-2-hydroxyglutaric aciduria) in Staffordshire Bull Terriers 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, or change to the structure of the gene, probably occurred spontaneously in a single dog but once in the population has been inherited from generation to generation like any other gene. The disorder shows an autosomal recessive mode of inheritance: two copies of the defective gene (one inherited from each parent) have to be present for a dog to be affected by the disease. Individuals with one copy of the defective gene and one copy of the normal gene - called carriers - show no symptoms but can pass the defective gene onto their offspring. When two apparently healthy carriers are crossed, 25% (on average) of the offspring will be affected by the disease, 25% will be clear and the remaining 50% will themselves be carriers

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.

L2HGA isn't restricted to staffords. You'd have to be really unlucky to find a stafford cross with it, but it's possible!

The easiest way to find out is to send a swab off to the AHT who can test it and see if that specific gene is present. It costs a bit, but it'll give you a little more peace of mind.

Have a look here....

http://www.aht.org.uk/cms-display/genetics_l2hga.html

The cost of the test is, I believe, £40.

Quote: L2HGA isn't restricted to staffords. You'd have to be really unlucky to find a stafford cross with it, but it's possible!

hahaha unlucky is exactly how I would describe our poor little guy. He has had a tumor removed from his side, has had seizures since before he was 2, is on medication for his thyroid, and other issues....unlucky is all we know.

CCSC wrote:
hahaha unlucky is exactly how I would describe our poor little guy. He has had a tumor removed from his side, has had seizures since before he was 2, is on medication for his thyroid, and other issues....unlucky is all we know.

Poor little guy!

Are the seizures really bad?

What's he on for the seizures?

Yes, the seizure can be bad. But in the last 2 years, he's been doing great. When he has them, they are shorter with less recovery time. I've done sooo much research and do everything I can for him that is suppose to help control his seizures, medication wise and natural.

For the seizures he is on Potassium Bromide, Phenobarbital and levetiracetam (keppra). He is also on two liver medications, as well as a medication for his thyroid. He also twitches a lot. He's very happy though, and has no idea something is wrong ) He's my love.

hahaha, that was suppose to be a smiley face!

He sounds really sweet - any chance of some pics?

I have no idea how to add pictures!

CCSC wrote:I have no idea how to add pictures!

Here ya go.....

https://staffy-bull-terrier.niceboard.com/t12017-how-to-post-a-photos-onto-the-forum

https://servimg.com/view/18382090/7

not sure if this worked....I don't have a photobucket account!

He's a real beauty, it's really much easier if you open a photobucket account which is quick & easy

Sorry to hear about the health issues .. sounds like your little man has had a tough time.

He is a gorgeous boy .. love the little dot near his tail

Thanks

He does have a tough time, but he's a very happy boy, so its all worth it.

I spoke with my vet, and we have decided not to test him. Being that he is already nine, neutered and not being bred, she explained that the treatment would be no different then what we are already doing. If we did test it would really be just for curiosity sake and not worth the money. We are doing everything we can for him, and will continue to do so.

Thanks for all who replied!

I think the test from AHT (animal health trust) is around £40 - defo worth it IMO - quick swab inside the cheek (u do it ur self) and result by email a few days later.