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.
There are no current data on how common the mutation is among U.S. Basset Hounds, but recent testing suggests that Lafora Disease may be more prevalent in the U.S. than previously thought. The mutation is being found in dogs and puppies with U.S. pedigrees, making clear that the Lafora mutation is NOT limited to dogs with European ancestry.
Testing is strongly recommended for all dogs for three reasons:
1) The late onset of this disorder means that
dogs with the mutation will likely be bred
before any symptoms become evident,
2) owners of dogs that test affected can take
steps to mitigate symptoms, and
3) widespread testing will provide valuable
information about just how common the
mutation is in the U.S.
The BHCA Health Committee WILL offer Lafora testing at the 2022 National Specialty. We have arranged testing at discounted rates for the four disorders for which we have verified genetic tests: MPS1, POAG (Primary Open Angle Glaucoma), Thrombopathia, and Lafora. Three of these tests are available from Paw Print Genetics and will be offered at the Nationals, along with their test for CDDY/IVDD (indicator of risk for intervertebral disc disease). Your dog will be swabbed just once, and you will be able to choose which tests you want run. The MPS1 test is only available as a home test through OFA, who will also offer the test at a discount. The discounts on both the Paw Print and OFA tests will be honored whether people test at Nationals or order the tests online between October 1st and November 30th, 2022.
We've had a number of questions about the specifics of testing (timing, cost, etc...) and we are still working on those answers but we WILL update you through FB, Constant Contact and the Nationals website as soon as we get the details tied down.
In the meantime, it is helpful for us to know if you wish to test your dogs at the Nationals, and how many you will have with you so that we can estimate the number of test kits we need. All four tests from Paw Print will be run from a single swab, making this process easy for you and your hounds. Drop a line to Rosemary Ryan (r2ryan46@wavecable.com), chair of the Health Committee, to let us know if you plan to test at the Nationals.
