Lafora Disease in Miniature Wirehaired Dachshunds

Miniature Wirehaired Dachshunds are predisposed to an inherited progressive disorder known as Lafora Disease. It is a type of epilepsy called myoclonic epilepsy which sees affected dogs experience a type of jerky"" seizure that are typically triggered by sudden sounds and movements as well as flashing lights which happen close to a dog's head or in their line of vision. The seizures can be partial, generalised or more complex and as time goes by, the symptoms gradually become more severe leading to a Miniature Wirehaired Dachshund eventually developing ataxia, dementia and total blindness.

It is worth noting that Lafora disease can affect any breed, but the most affected are the Miniature Wirehaired Dachshund along with the Bassett Hound as well as Beagles all of which are the most at risk of inheriting the disorder. Dogs usually first start to show signs of there being something wrong with them when they are anything from 5 to 7 years old and both males and females can be affected by the condition.

Signs of there being something wrong with a Miniature Wirehaired Dachshund

As previously mentioned, signs of there being something wrong with a Miniature Wirehaired Dachshund usually manifest themselves when a dog is anything from 5 to 7 years old. The first indications of there being a problem are as follows:

• A group of muscles suddenly start contracting
• Dogs will suddenly start jerking or shuddering their heads backwards

The disorder happens spontaneously when any sort of noise, flashing or flickering of light as well as sudden movement happens close to a dog and more especially in their line of sight. Flickering that often occurs on televisions can be the trigger too.

Hypnic (sleep related) myoclonus

Some Miniature Wirehaired Dachshunds can also suffer from Hypnic myoclonus which is sleep related and some dogs may also suffer epilepsy as a consequence of having developed Lafora Disease. The episodes can be either generalised ""tonic clonic"" seizures or they can be complex ""partial"" seizures. When a Miniature Wirehaired Dachshund has more complex seizures, they often become quite vocal and appear panicked during an episode.

Diagnosing the condition

A vet would need to have a dog's full medical history and be told how the onset of any symptoms first presented themselves and when they first occurred. The vet would need to carry out haematological and biochemical tests with the end goal being to eliminate any other reactive reasons why a dog might be suffering from epileptic seizures. Other tests a vet would want to carry out to confirm a diagnosis includes the following:

• Magnetic resonance imaging - this test helps identify if a dog has developed any sort of structural brain disease which could be the reason for them experiencing acquired epilepsy

It is worth noting that dogs typically suffer from idiopathic epilepsy when they are that much younger which is usually when a dog is anything from 6 months to 6 years old and when this is the case, myoclonus is not part of the problem.

The diagnosis can also be confirmed by establishing if any Lafora bodies are present in a dog's liver, their muscles and by carrying out a nerve biopsy.

How the genetic mutation in Miniature Wirehaired Dachshunds was identified

The actual gene mutation responsible for Lafora Disease in Miniature Wirehaired Dachshunds was first discovered in The Hospital for Sick Children in Toronto Canada and the official club for the breed then ran a national screening programme with an end goal being to eliminate the disorder altogether.

The causes and risks

The cause of the disorder, as previously mentioned is a genetic mutation which is known as EPM2B (NHLRC1) which is basically the protein that takes care of metabolising carbohydrates which then make sure not too many of them build up in a dog's system. When too many toxic materials which are starch-like in nature build up in nervous and liver cells as well as in a dog's muscle tissue, it results in them developing the disorder.

Lafora Disease is inherited by what is known as an autosomal recessive method which in short means that both parent dogs must carry the mutated gene for offspring to inherit the condition even though they don't show any signs of having the disease themselves and just have one copy of the mutated gene or show symptoms of suffering from Lafora Disease.

The condition is often aggravated by anxiety and stress which means keeping a dog quiet and in a calm environment goes a long way in reducing the risk of them suffering an episode.

Living and managing the condition

Once a Miniature Wirehaired Dachshund is diagnosed as suffering from Lafora Disease, they do not necessarily need to be put on any sort of medication unless they frequently experience seizures and if their head shuddering/jerking is so bad that therapy becomes essential. Sometimes dogs with the condition do not respond well to any sort of traditional treatments too and as such a vet would have to decide on which treatment would be the best for any individual dog known to suffer from Lafora Disease and would typically refer a dog to a veterinary neurologist.

What about diet?

Because Lafora Disease is associated with too much starch-like material being stored in a dog's cells and muscle tissues, studies suggest that diet could play an important role in managing the disorder. As such, a dog's condition has been seen to improve when they are fed a low GI diet which in short means it is low in simple carbohydrates like starch and glucose and as such it is best to feed a dog with Lafora Disease a low grain diet. It is also worth noting that sugary food treats may make a dog's condition that much worse. It is also essential to discuss things with a vet before altering a dog's diet and to follow their advice on what to feed a Miniature Wirehaired Dachshund suffering from Lafora Disease.

What about dogs wearing glasses?

Studies have also suggested that putting sunglasses on dogs when they are being walked in bright sunlight helps improve things when it comes to head jerking. The sunglasses have become known as ""doggles"".

Prognosis

Lafora Disease is not a life-threatening condition, but with this said over time the seizures often get worse with many older dogs developing ataxia and eventually going totally blind.

The genetics of the disorder

For a Miniature Wirehaired Dachshund puppy to inherit the disease, they would have inherited a copy of the mutated gene from both of their parent dogs. The genetically inherited mode for the disorder is as follows:

• Both the dam and the sire would be a ""carrier"" and have one copy of the mutated gene therefore not affected themselves
• Both the dam and the sire would be ""affected"" and as such both have a copy of the mutated gene which they both pass on to their offspring

All offspring produced by a Miniature Wirehaired Dachshund suffering from Lafora Disease would either be ""carriers"" or ""affected"" and therefore should not be used for breeding purposes.

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