LAFORA EPILEPSY IN BEAGLES

Lafora disease is a recessive genetic trait inherited late onset progressive myoclonic epilepsy. Myoclonus (jerking) is a feature of the disease which characteristically can be induced by bright sunlight, flashing lights, sudden sounds and movement especially that is close to the dog's head. Generally, the clinical signs of this brain disorder appears at 5-6 years of age or later. It progresses, gradually causing neurological changes over many years. These neurological changes include mainly myoclonus/jerking (sudden involuntary muscle jerking or twitching typical for epilepsy) The frequency of seizures increases over time and the uncontrolled jerking and twitching is followed by other neurological symptoms such as ataxia, twinkling, blindness or dementia. During seizures, the convulsions are commonly accompanied by muscle rigidity, vocal utterance, salivation, urination or loss of consciousness. The epileptic seizures can arise in spontaneous unpredictable fashion or can be induced by light flashes, sudden sounds or movements, particularly movements close to the dog's head.

Lafora has been discovered in pedigreed miniature wire-haired dachshunds. Other breeds suffering from this disease include Basset Hounds, Miniature and Standard Poodles, Pointers, Welsh Corgis and Beagles. This form of epilepsy is incurable and is fatal. The therapy is currently limited to treatment of seizures; suitable diet and medication prescribed help to keep the seizures under control.

A NHLRC1-gene  mutation is responsible for Lafora's epilepsy in beagles and 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 effects. Besides the brain tissue, the Lafora bodies are found in the muscles, skin, liver and heart. Lafora disease is actually a glycogen metabolism disorder that demonstrates itself as progressive myoclonus epilepsy.

Mutation that causes Lafora 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 symptoms. They are genetically considered carriers of the disease (heterozygotes). In offspring of two heterozygous animals the following genotype distribution can be expected: 25 % N/N (healthy non-carriers), 25 % P/P (affected), and 50 % N/P (healthy carriers).

Genetic testing to determine a dog's genotype is generally recommended. It can help the breeder to select potential breeding pairs to ensure that the risk of producing affected puppies is very low and this disease will be gradually eliminated from the breed. Listing of beagles tested for Lafora.
Here is a link to labs that offer the test for Lafora. Genomia Pet Genetics Lab-UK

TREATMENT OPTIONS

Research has shown that benefits may be obtained by a dietary change for you dog. These changes may include a change to foods with a low glycemic index, Starchy and sugary treats are suspected of aggravating the condition Various medications or combination of medications used in treating the epilepsy are phenobarbital, potassium bromide or levetiracetam (Keppra). Since sunlight is a known trigger for the seizures, some dog owners use doggy sunglasses when they have their family pet outdoors during walks and other exercise activities. Also, you may need to be more aware of the flashing lights in your dog's environment that might trigger seizures. One example would be lights which emanate from the television during some programs are video games.

ADDITIONAL RESOURCES

You Tube videos of affected beagles:
https://www.youtube.com/watch?v=dgKTKV2CdGo
https://www.youtube.com/watch?v=t7LYu9uX1yY Https://www.youtube.com/watch?v=JzysRR3hP-M&t=27s
https://www.youtube.com/watch?v=mg_m8WmWWvc&t=28s

Informational Websites:

http://www.beaglehealth.info/genetics/lafora.html
http://www.veterinary-neurologist.co.uk/Laforas_disease/
https://wagwalking.com/condition/lafora-disease http://beaglehealth.info/genetics/lafora.html


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