Genetic Testing is Available for a Newly Discovered Disease in Basset Hounds

By Don Bullock, BHCSC Webmaster and former club President


We have a serious disease, Lefora, that has recently been brought to the our attention that affects all basset hound breeders, owners and those seeking to get a puppy. It’s something that all basset hound lovers should know about as well. Fortunately Lefora something we can test for.

Rudi Smits, a well known basset hound breeder and  veterinarian at Veterinary Clinic Den Heuvel in Nuland, Netherlands broke the news about Lefora in basset hounds on Facebook sometime in 2021. I’ve searched but can’t find the Facebook post where Rudi reported the test results on one of his bassets. Since then many breeders and the Basset Hound Club of America have been diligently working on getting the word out to breeders about the disease and making genetic testing for Lefora available here in the United States.


This writer greatly appreciates the fact that Rudi Smits stepped up and quickly let all his many Facebook followers know about the test results on his dog and the disease. Without honest breeders like him we would not have known about the disease or at least wouldn’t have known as quickly as we did.


It’s been brought to my attention that some have started blaming breeders like Rudi for this situation stating that they should have known about the disease. That’s absolutely not what we should be doing. No one knowingly has bred bassets that were affected by Lefora or knowingly bred carriers of the disease. Until Rudi boldly spread the word none of the breeders had heard anything about Lefora.


Many of those who’s basset hounds may have been affected due to this one basset’s test results have been testing their bassets. As the results started being reported others began having their bassets tested as well. This was difficult for those in the United States to do because the only testing sites available at the time were in Europe. Sometimes the samples being sent to the lab never made it.


Now, thanks to basset hound breeders here in the United States, the Basset Hound Club of America and the BHCA Health Foundation, we do have testing available in this country. As more basset hound owners and breeders have their bassets tested we’re starting to discover that Lefora is much more widespread than was expected. At this time we still don’t have any accurate statistics for the United States but those should be forthcoming fairly soon because a large number of basset hounds are currently being tested as I write this blog.   


The following is a quote regarding Lefora and the testing taken from the BHCSC website:


Lafora Disease


Lafora Disease is a late-onset (5-7 years of age) inherited neurological disorder affecting a number of breeds including the Basset Hound. The disease is caused by a mutation which produces intracellular accumulation of abnormal glycogen (Lafora bodies). Symptoms are varied and include a characteristic quick and involuntary jerking of the head backward (myoclonus), jaw smacking, panic attacks, impaired vision, loss of balance, aggression, and urinary incontinence. Symptoms can occur spontaneously or be triggered by sudden sounds or movements, or flashing/flickering light, and can progress to partial or generalized epileptic seizures. Diet and medication are thought to help in controlling seizures, but Lafora is ultimately fatal.

Two laboratories in Europe offer tests for it. This past year, several BHCA members had roughly 100 U.S. dogs tested at these labs and a high percentage of dogs were found to be carriers or genetically affected. Some have developed clinical signs.

Neogen/Paw Print Genetics has been working on a test for Lafora for several years. With samples from carriers and affected dogs sent by BHCA members, they were able to complete test development. Starting October 1, 2022, we will have a validated an accurate cheek swab test in the U.S.! Tests can be ordered from Paw Print Genetics at www.pawprintgenetics.com.

NOTE: To see the original posting of this information on the BHCA website and additional important health information on basset hounds go to: https://basset-bhca.org/about-bassets/health-research/

 

Obviously this is very important information for basset hound breeders, basset hound owners and those who are considering getting a basset hound puppy. The only way to eliminate this disease is to have bassets tested, especially those that are being bred. Every basset hound breeder needs to get involved in testing their breeding stock. Basset hound owners, especially those who’s bassets have shown some of the symptoms mentioned above may wish to have their bassets tested so they can be more informed about their dogs health.

Most importantly people who are interested in getting a basset hound puppy should insist that both the mother (dam) and father (sire) of the litter be tested and they can see the official results. Since Lafora Disease is inherited in an Autosomal Recessive manner in dogs, meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease, it’s important that only one parent of a litter is a carrier, not both. Until breeders have worked on ridding our breed of this disease it may be common that one of the parents is a carrier based on the preliminary testing results we have at this point in time. 

Reliable genetic testing is important for determining breeding practices. Because symptoms do not appear until adulthood, genetic testing should be performed before breeding. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Dogs that are not carriers of the mutation have no increased risk of having affected pups.

This kind of issue, and the other health issues that basset hounds can have is the main reason I always advise those who are looking for puppies check with reputable/responsible breeders. They are more likely going to follow their breed’s parent club’s (i.e. Basset Hound Club of America) health policy when breeding their litters. Unfortunately that may not always be the case. It’s important for anyone seeking puppies to do their part in asking questions about the health testing that’s been done on the parents of any litter and insisting on seeing the official results not just taking a breeder’s word. There are far too many breeders who aren’t aware of this disease or who do not do any health testing or screening. 

In the past we didn’t have as much accuracy in the testing of basset hounds but that has improved in recent years. Fortunately for basset hounds the Basset Hound Club of America and the nonprofit Basset Hound Health Foundation have been hard at work trying to research health issues in the breed and come up with some reliable testing that can be administered. There still needs to be a lot more done in this area but at least in the case of Lefora we actually have a genetic test that should help eradicate it from the breed. Now it’s up to breeders and those looking for puppies to BOTH do their part in making that a reality.