What is Canine Lafora?

Canine Lafora shares many similarities with the human form of the disease. It was first identified as the result of a research programme into teeneage lafora. You can read more about the reseach here  , find out how to get your dog tested or download a copy of the Dachshund Breed Council Dach-Facts Lafora leaflet to pass on to friends or vets.

Symptoms

One of the most typical symptoms of a Lafora affected dog is myoclonic ‘jerking’ , particularly in response to flashing lights, noises or movements close to the head. The condition can worsen over several years and in time they may also suffer from epileptic seizures, blindness, ataxia (unsteadiness) and dementia. The condition is not fatal in dogs, but in due course, most owners will have to take the ultimate decision as their dog’s quality of life deteriorates. A short video of myoclonic jerking in a Lafora affected dog can be viewed on Dr Clare Rusbsridge’s Lafora webpage here, or watch  Keira, Laforadog, exhibiting typicalLafora jerking here  or Alfie, Laforadog, demonstrating difficulty with blindness and dementia here, and ataxia here and read more about owner experiences in our Blogs on Dogs section.  There is currently no cure for Lafora.   Owners of lafora dogs have experimented with various management strategies to cope with symptoms - read here for more. Put as simply as possible (!), Lafora is an inherited disorder, caused by the mutation of certain genes which are linked with the processing of carbohydrates into glycogen. Glycogen is used by all animals as an energy store. In Lafora affected humans and dogs, some of the carbohydrate conversion to glycogen does not happen normally but instead, ’Lafora bodies’ are formed, which are made up of a form of abnormal glycogen called polyglucosan. These starch-like polyglucosans are insoluble and can end up inside cells all over the body, including the brain and central nervous system. This accounts for the epilepsy like symptoms, the ‘myoclonus’ or jerking, sensitivity to light and sudden noise, apparent blindness and dementia that characterises the condition as it progresses.

The disease particularly affects the miniature wire-haired dachshund, bassett hound and beagle. Typically signs first appear in animals over 5 years age and both sexes can be affected.  Whereas it is inevitably fatal in humans, , it is more common that the ultimate decision of euthanasia is taken for the dog by the owner when the the quality of life of the dog is no longer acceptable.

My dog doesn’t show any sign of Lafora, so why is it important to me?

Because the disease is an ‘autosomal recessive’ genetic disorder, the condition only manifests itself when a lafora gene is inherited from each parent. If a lafora gene is only inherited from one parent, then the animal will not show any sign of the disease, but it may pass that gene on to any offspring.

In humans the disease rapidly progresses in teenage years which means it is less likely that the faulty genes will be passed on to any child. It is, thankfully, a very rare human condition.

However, in dogs, because the disease does not manifest itself until ‘middle age’, affected dogs may have been used for breeding from around 1 year old which means that many puppies could have been produced. All of these will be carriers. If they in their turn are bred to other carrier dogs, then more affected dogs will be born. It is easy to see in these circumstances how quickly the condition can spread, particularly amongst a restricted population of pedigree dogs where there might have been significant inbreeding in the past.

This diagram:  Lafora genetics RAG chart April 2011 graphically illustrates how this works and reinforces the importance of knowing the status of any dog before breeding. This is why the Wirehaired Dachshund Club have initiated a 3 year UK testing programme in order to identify exactly which dogs are affected, are carriers and are clear. Whether you breed or are a pet owner, we need your support to build a database of information on this terrible condition.  To find out more, whether you are UK based or not, go to how to get your dog tested.

If you are thinking of buying a mini wire, please read the attached first – you can print this off double sided, so keep one for yourself and pass one on to a friend. If you would like hard copies, contact me at gillkey@bitnternet.com.

 

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