Lafora Disease in Dogs

As noted above, Lafora disease is a myoclonic epilepsy, meaning that it presents with jerking motions as its primary characteristic.  Generally, it comes on spontaneously after about the age of 5 years and is not gender specific.  While it can afflict any breed of canine, it seems to be most often found in the Miniature Wirehaired Dachshund, Basset Hound and Beagle breeds, with the Beagle breed seeming to suffer more severe cases of it. It is progressive, gradually causing neurological changes over many years which can include ataxia (lack of muscle control), blindness and dementia.

Lafora disease is simply defined as a myoclonic (jerking) epilepsy which is inherited and generally presents as a late onset and progressive type of epilepsy.

These are the symptoms of Lafora disease in dogs that one might see in a canine who suffers from the seizures:

• Rapid shuddering or shaking or jerking of the head of the canine backwards
• High pitched vocalizations or other behaviors to signal that the dog might be panicking
• Seizures
• Later in disease, dementia, blindness and loss of balance may occur

There are not many symptoms for this disease and they will come on suddenly as a result of some outside stimuli noted below.

Types  

Lafora disease is a rare type of inherited epilepsy in canines which can occur in any canine breed but seems predisposed to Miniature Wirehaired Dachshunds, Basset Hounds and Beagles.  The Beagle breed seems predisposed to a more severe type of Lafora disease than other canine breeds and it seems that the Beagle variety of the disease is more drug resistant as well.  The disease generally develops after the age of 5 years and is a gradually progressive disease which can lead to ataxia, blindness and dementia, just to name a few neurological changes for which the disease can be responsible.

Lafora disease seems to be caused by a genetic deviation which prevents the manufacturing of a specific protein which is believed to play a rather significant role in the “cleaning up” of other proteins which are no longer needed by the cells in the brain.  It seems to be the buildup of these no longer needed proteins which cause the cells in the brain to experience “interference” with their function.  While this is obviously a very simple description of what is happening within a very complex system, the meat of this is that fact that it's in the DNA of your family pet and there is no test currently available which is designed to detect the presence of the gene before it presents in the canine.  There are recommendations about what things you might wish to avoid if you are a pet parent of a canine in any of the predisposed breeds.  Here are some of the stimuli which seem to trigger the episodes:

• Flashing lights - Like strobe lights or even lights from the TV in your home
• Sudden sounds - Sounds unexpected by the canine
• Sudden movements - This is especially true of sudden movements occurring close to their heads

Your veterinary professional will require a thorough history of your pet so you might want to be prepared to provide answers to questions from your vet regarding dietary regimen, exercise activities, housing accommodations and any symptoms you have noted along with the severity, frequency and duration of those symptoms. It might be helpful to know a bit about the familial history of your canine as well.   Your vet will perform a physical examination and will likely order blood work and some tissue samples for laboratory evaluation.  

Even though the disease causing genetic mutation has been identified in the Miniature Wirehaired Dachshund and the Basset Hound through much research, there is no commercially available diagnostic test to identify it in your canine family member.  The only definitive method of diagnosing Lafora disease in your pet will need to come from the identification of Lafora bodies found with microscopic evaluation of liver, muscle or nerve biopsies.  Your vet may also require an MRI of the brain to rule out other potential differential diagnoses which are also known to cause seizures.

The treatment options for Lafora disease in dogs can include:

• Dietary changes may include a change in the food you are feeding your canine family member as some research has shown that foods having a low glycemic index are beneficial to some canines suffering from seizures; starchy and sugary treats are suspected of aggravating the condition
• Administration of various medications with the intention of treating the epilepsy; these drugs might include phenobarbital, potassium bromide or, in cases when these have not worked successfully to control the seizures, trying levetiracetam (Keppra) which may actually work better than phenobarbital in some canine epilepsy situations 
• Changes in some of your canine’s lifestyle activities as sunlight is a known trigger for the seizures and some dog parents have opted to use doggy sunglasses (Doggles) when they have their family pet outdoors for walking and other exercise activities
• Along the same lines, you may need to be more aware of the flashing lights which emanate from the television during some programs

Lafora disease in dogs, though a rare inherited disease, is not generally fatal for your pet.  It will, however, likely cause significant debilitation to your canine family member as the disease progresses. It is this debilitation which frequently brings the parents of a Lafora disease afflicted canine to a decision concerning possible euthanasia. The use of Doggles (doggy sunglasses) can help reduce the jerking in some dogs and could be an option when your canine is outside.  

Balancing medication dosing, moderate levels of exercise, diets and avoidance of the known triggers of the seizures can be challenging but will be so worth it in terms of maintaining the best and safest lifestyle and environment for your canine family member.  This is an area where extra measures of the three A’s -- affection, affirmation and attention -- would really help ease your pet through the inevitable progressive process of this disease.