Lafora Disease

Article written by: Gill Key, Laforadogs coordinator
(with thanks to Clare Rusbridge, Veterinary Neurologist)

 

Lafora Disease is an inherited form of epilepsy which can occur spontaneously in any breed of dog, but it particularly affects the miniature wire-haired dachshund, bassett hound and beagle. Myoclonus (jerking) is a
feature of the disease and characteristically this can be induced by flashing lights, sudden sounds and movement (especially that close to the dog's head). Generalised or complex partial seizures may be seen in some
dogs. The disease progresses slowly over many years and gradually other neurological deficits such as ataxia, blindness and dementia occur. Dogs may have a near normal life expectancy, though as quality of life
diminishes, the owner may be forced to take the ultimate tragic decision. Typically signs first appear in animals at any time after 5 years of age and both sexes can be affected.

In 1995 UK veterinary neurologists identified that dogs exhibited similar symptoms to those shown by human teenagers suffering from Lafora, one of the most severe (and fatal) forms of human epilepsy. Blood samples taken from UK dogs helped identify the gene causing the problem in both cases, and since
then it has been confirmed that the condition is due to an inability to metabolise carbohydrates correctly. As a result, insoluble starchy bodies are deposited in the central nervous system. There is some evidence to
suggest that reducing the amount of carbohydrate in the diet (low GI), e.g. by choosing non-cereal based foods may reduce the speed of the build of these bodies.

Tests in 2010/11 indicate that the number of affected dogs in the UK may be as high as 12% which would in turn indicate that anything up to 60% of dogs carry at least one copy of the gene. Because there are a small number of very successful breeders who have produced many champion dogs in the UK, inbreeding has been very common in the past, but the majority of breeders are now behind efforts to introduce a UK wide testing programme to reduce the incidence of dogs being born with the condition.

To find out more about the condition and the testing programme go to www.laforadogs.org.

Gill Key, Laforadogs coordinator (with thanks to Clare Rusbridge, Veterinary Neurologist)

Page last update: 07/18/2011