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Lafora is a later onset Epileptic Seizure Disorder in dogs and Humans. Nearly always fatal because of the symptoms , progression and lack of treatment options. It was never know as an issue in basset hound until recently., and preliminary genetic testing is show I high rate of carriers and affected genetically however symptomatic dogs are far fewer whether this is because it is relatively new occurrence in the breed or that not all genetically affected will developed the disease is not well understood. "Canine Lafora disease is caused by a dodecamer repeat expansion mutation in the NHLRC1 gene and a DNA test is available to identify homozygous dogs at risk, carriers and dogs free of the mutation. "1 the expansion is variable and it is speculated the larger the expansion the Higher the risk of actual developing the disease but this speculation. There coming Oct 1 2022 Pawprints Genetic Has developed a check swap test for Basset hound A discount is available from, Pawprints if order only by November 30th 2
or the Lafora test, use the code word BHCAL.
For all other tests, place a separate order using the code word BHCAHC22.
this is far cheaper and easier than the two available test in Europe developed for different breed but given the same exact gene is involved the test is accurate in basset hounds as well

1. Nationwide genetic testing towards eliminating Lafora disease from Miniature Wirehaired Dachshunds in the United Kingdom - Canine Medicine and Genetics

2. https://basset-bhca.org/wp-content/...-Health-Issue-Bulletin-V3.2-8.2022-Lafora.pdf

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"Currently, there is no curative treatment for Lafora disease, and disease management is limited to controlling the myoclonus and seizures [27]. Levetiracetam can be useful to reduce the myoclonus in the early stages of the disease and was used to reduce myoclonus in Cases 1, 2, 4 and 5. Levetiracetam is a new antiepilepsy drug with an antimyoclonic effect, especially when the myoclonus is cortical in origin [28,29] ...The evidence of increased oxidative stress due to a glycogen storage disease means that many veterinary surgeons recommend a proprietary antioxidant-rich, low-carbohydrate diet in the management of Lafora disease [35]. However, there is poor evidence that this alters the disease course [5]. A lifelong ketogenic diet reduces Lafora bodies in a mouse model of Lafora disease; however, human studies were less promising but did suggest that the disease may progress more slowly if a ketogenic diet was started early in the disease process [36,37]. A ketogenic diet aims to maintain blood glucose concentration in the low normal range and the brain uses more keto acids for energy production. A ketogenic diet is also antiepileptic due to γ-glutamyl transpeptidase and γ-glutamylation inhibition [37]. There is one commercial medium-chain triglyceride ketogenic diet (Purina Neurocare™) that has achieved clinically meaningful levels of ketosis in dogs and helped prevent seizures and improve cognition in dogs with idiopathic epilepsy [38,39,40]. This diet has not been trialed for Lafora disease, but based on the available evidence, this or a similar diet is probably the most appropriate for dogs with Lafora disease. However, not all dogs or owners accept a change in diet, especially if the perceived benefit is not obvious or unproven.
"Lafora Disease has been recognized in Basset Hounds for years, but prior to last year most of us had never heard of it. Signs usually appear between 5-7 years of age and progress to epileptic seizures. 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 and accurate cheek swab test in the U.S.!

...There is much we do not yet know about Lafora. The relationship between the mutation and the clinical presentation of the disease is confusing. On the one hand, we know the disease-causing mutation for Lafora has been identified in the breed for some time. Yet very few people report seeing seizures in older dogs. Tests of U.S. dogs done in European labs suggest the mutation is widespread, yet few dogs show clinical signs. Perhaps there is a recent upsurge in the number of dogs with the mutation or perhaps not all dogs that are genetically “affected” actually develop disease. For the sake of the breed, we need to know: • What percentage of Basset Hounds carry the mutation and how many are genetically affected, and • Of those with two copies of the mutation, how many go on to develop clinical signs.
 

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On a personal note we Know that FC Soundtrack Pandoras Box NAP NJP is a carrier without actual genetically testing Two off spring and the Sire of a recent litter were recently test in using an English Lab. On of the off spring was clear, one was a carrier and the Sire was clear. Meaning Hope is a Carrier. Eagerly await the result for baker because being four years old there were no genetic test at the time both his Sire and Dam are carriers so he has a 25% chance of being an affected. Being a singleton we do not have to worry about littermates. We have tested all our younger potential breeding dogs and go from there. In breed that have testing in realetively short time they have made dramatic inroads in reducing the prevalance without destroying genetic diversity.
 

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Interesting - and I'm more than glad I'm no longer breeding Bassets! I ended my bloodline because an outside sire of what was to be our last litter developed epilepsy when aged 8+. Obviously long after we'd used him but it turns out that his mother was also lost to epilepsy when about the same age as her son was. As a result, I had the bitch we'd kept from what was an excellent litter, spayed without bred from, and her brother was never used at stud despite attracting some interest which I turned down. I felt the buck had to stop somewhere - others had used the same stud dog but I don't think he's behind many lines, if any, these days. Neither of the hounds I kept from that litter developed epilepsy - both were lost to different forms of cancer in old age. I don't think any of those we sold from that litter as pets did either, and if I'm honest, I would have heard from their owners had that been the case.

Was this what that stud dog had - much as indeed epilepsy it known within the breed here. Thought to have come from the outcrosses to Bloodhounds.
 
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