What causes Lafora disease?
Most cases of Lafora disease are caused by changes ( mutations ) in either the EPM2A gene or the NHLRC1 gene. These genes encode proteins that play a critical role in the survival of nerve cells (neurons) in the brain.
What are the symptoms of Lafora disease?
The first 2–3 years of Lafora disease are characterized by the following symptoms:
- Ataxia.
- Confusion.
- Depression.
- Grand mal seizures.
- Staring spells and/or absence seizures.
- Drop in school performance.
- Drop attacks.
- Myoclonus.
Is myoclonic epilepsy fatal?
Progressive. Progressive myoclonus epilepsy is a disease associated with myoclonus, epileptic seizures, and other problems with walking or speaking. These symptoms often worsen over time and can be fatal.
What causes Sialidosis?
Sialidosis is caused by mutations of the NEU1 gene. This gene mutation is inherited as an autosomal recessive trait. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother.
What triggers myoclonic seizures?
Myoclonic seizures are caused by abnormal electrical activity in the brain, which triggers the myoclonic muscle movements. Often, they are exacerbated by tiredness, alcohol, fevers, infections, photic (light) stimulation, or stress.
Can a dog with Lafora’s disease be killed?
Lafora’s disease is not generally fatal in the dog however the myoclonus and seizures may worsen with time and older dogs may become blind and ataxic compelling the owner to request euthanasia. The beagle has a different genetic mutation and a more servere form of the disease.
Why is there a problem with Lafora’s disease?
Lafora’s disease is due to excess storage of a starch-like compound, polyglucosan in the nerve cells. Latest research suggests that in Lafora’s disease there is a problem with the control of the manufacture of glycogen and/or regulation of insulin sensitivity.
Can a dachshund have Lafora’s disease?
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. Lafora’s disease can occur spontaneously in any breed however the miniature wire-haired dachshund, Bassett hound and beagle are predisposed.
What kind of epilepsy does Lafora’s dog have?
Lafora’s disease is an inherited, late onset, progressive myoclonic epilepsy. 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).
