Lafora Disease in Mini Wires – the sad reality

On the day that the WHDC Lafora Team felt it necessary to issue this statement about the screening programme, I was saddened to read about the sudden death last night of an 8 year old Mini Wire who died following an epileptic fit.  She had been diagnosed with Lafora Disease and her responsible owner had had this confirmedby making use of the available DNA test.  I hope that the numerous messages of sympathy will be of some comfort to her owner at this difficult time.

Lafora Disease is a late-onset form of Epilepsy found in Mini Wire Dachshunds and is caused by an autosomal recessive mutation. The gene was identified in 2005 and an associated DNA test was developed and reported in a peer-reviewed paper, with patents filed in 2007 and 2009. The KC agreed to recognise this as an “Official Test” earlier this year and results are now being published in the Breed Records Supplement.

The WHDC has been using the Canadian Laboratory that developed the DNA test since 2010 and now has full-spectrum (Clear/Carrier/Affected) results from 276 dogs. Of those, it has been found that 7.9% of dogs are Affected, 38.7% are Carriers and are 53.9% Clear.

Please do not be misled by anyone who says this is not a problem in the breed. In the Breed Council’s 2012 Breed Health Survey the clinical prevalence of Lafora in dogs over the age of 5 was 7.6%.  Given the margin of error for this survey sample, we can be confident that the population prevalence of Lafora Disease lies in the range 5-10%.

Veterinary advice and the interim results of the current Lafora Progression Survey indicate that the majority, if not all “Affected” dogs, will go on to develop clinical symptoms over time, including debilitating myoclonus, ataxia, vision impairment and perhaps most distressing of all, dementia.

The KC Charitable Trust donated £17000 to help scale-up and implement the test over the past two years. Our Breed Clubs have donated around £25,000 to enable breeders to screen their dogs at a subsidised rate.

There is good news to report based on the Lafora Screening Results being published in the latest KC Breed Records Supplement.  As a result of more breeders (ABS and non-ABS) adopting the test and using Clear dogs, 78% of litters (74% of puppies) bred in the first Quarter of 2014 are now in the safe category (with no Affected puppies), compared with 57% puppies at the end of 2013. We track and publish this information quarterly. It is also interesting to note that there were no affected dogs in the last sample batch, indicating that our education and testing programme is working.

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The advice from the Wirehaired Dachshund Club is simple:

If you own a Mini Wire, do not breed from him or her unless you have had the DNA test done and know the status.

If you are looking to buy a Mini Wire puppy, do not buy from anyone who cannot show you the DNA test results of both the parents.

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