LAFORA EPILEPSY IN BEAGLES
is a recessive genetic trait inherited late onset progressive
epilepsy. Myoclonus (jerking) is a feature of the disease which
characteristically can be induced by flashing lights), sudden sounds
movement especially that close to the dog's head. Generally, the
appear at 5-6 years of age or later. It progresses, gradually
neurological changes over many years. They include mainly
(sudden involuntary muscle jerking or twitching typical for epilepsy)
frequency of seizures increases over time and the uncontrolled jerking
twitching is followed by other neurological symptoms such as ataxia,
blindness or dementia. During seizures, the convulsions are commonly
accompanied by muscle rigidity, vocal utterance, salivation, urination
of consciousness. The epileptic seizures can arise in spontaneous
fashion or can be induced by light flashes, sudden sounds or movements,
close to the dog´s head.
It has been discovered in pedigree miniature wire-haired dachshunds. Other breeds suffering from this disease belong can be Basset Hounds, Miniature and Standard Poodles, Pointers, Welsh Corgis or Beagles. This form of epilepsy is incurable and fatal. The therapy is currently limited to treatment of seizures; suitable diet and medication help to keep the seizures under control.
A NHLRC1-gene mutation is responsible for Lafora´s epilepsy in beagles und miniature wire-haired dachshunds. The gene dysfunction leads to aggregation and accumulation of polysaccharides and formation of glycogen particles (named Lafora bodies). Simply, the NHLRC1-gene takes part in protection of tissue against carbohydrate accumulation. The protective power is lost due to this mutation. The Lafora bodies are gradually growing in the central nervous system where they have neurotoxic effect. Besides the brain tissue the Lafora bodies are found in muscles, skin, liver and heart. Lafora disease is actually a glycogen metabolism disorder that demonstrates itself as progressive myoclonus epilepsy.
Mutation that causes Lafroa epilepsy is inherited as an autosomal recessive trait. That means the disease affects dogs with P/P (positive / positive) genotype only. The dogs with P/N (positive /negative) genotype are clinically without any symptom. They are genetically considered carriers of the disease (heterozygotes). In offspring of two heterozygous animals following genotype distribution can be expected: 25 % N/N (healthy non-carriers), 25 % P/P (affected), and 50 % N/P (healthy carriers).
Genetic testing that enables to determine the dog´s genotype is
recommended. It can help the breeder to select potential breeding pair
ensure that the risk of producing affected puppies is very low and
will be gradually eliminated from the breed.
Here is a link to one lab that does test for Lafora. Genomia
Research has shown that benefits may be obtained by a dietary change for you dog. These changes may include a change to a food with a low glycemic index. Starchy and sugary treats are suspected of aggravating the condition Various medications or combination of medications use in treating the epilepsy are phenobarbital, potassium bromide or, levetiracetam (Keppra). Since sunlight is a known trigger for the seizures and some dog owners use doggy sunglasses (Doggles) when they have their family pet outdoors for walking and other exercise activities. Also, you may need to be more aware of the flashing lights in home environment that might trigger seizures. One example would be lights which emanate from the television during some programs are video games.
Tube videos of affected