Lafora Disease

What is Lafora Disease?

Lafora disease is an inherited form of epilepsy that can affect any breed but is known to be present in Miniature Wire-Haired Dachshunds, Beagles and Basset Hounds.

Find out what the Lafora symptoms are, making changes to your dogs diet to ease symptoms, and breeding advice for Lafora affected dogs.

What are the symptoms of Lafora Disease?

Lafora symptoms appear at any time from age 4 upwards, but typically around 6-7 and are progressive over time. First noticeable signs include one or more of:

• Generalised seizures
• Complex partial seizures (dog appears absent or loses the ability to move temporarily)
• Myoclonus (jerking back as if startled – some owners/vets initially misdiagnose as behaviourally linked)
• Ataxia (unsteadiness)
• Odd gait (stiff -legged, hopping, bouncy)
• Vision impairment (difficulty in focusing)
• Dementia
• Sleep disturbance/unsettled behaviour at night (to be confirmed)
• Incontinence – faecal and urinary
• Aggression – to dogs and/or humans
• Panic attacks – high pitched screaming as if in terror of unknown enemy, hugely fearful, does not recognise owner

Myoclonus (jerking) is one common feature of the disease (often induced by flashing lights, sudden sounds and movement close to the dog’s head). Generalised or complex partial seizures where the dog appears temporarily “absent”, ataxia (unsteadiness), blindness and dementia, including panic attacks, aggression to other dogs/owners, and incontinence can also be symptomatic.

Depending on the severity of symptoms dogs may die prematurely, or live to a ‘normal’ old age.

Lafora’s disease can occur spontaneously in any breed however the miniature wire-haired dachshund, Bassett hound and beagle are predisposed. Typically the first signs occur in animals over 5 years (average 7.5 years) age and both sexes can be affected.

The beagle has a more severe version of the disease and the associated epilepsy can be drug resistant.

A layman’s explanation of why the symptoms occur:

Dogs cannot process starches into sugars efficiently. Lafora results in a build up of insoluble platelets (Lafora bodies) inside cells all over the body, including the central nervous system and brain, which means they interfere with the neurological system.

The most common progressive myoclonus epilepsies are the late infantile and late infantile-variant neuronal ceroid lipofuscinoses (onset before the age of 6 years), Unverricht-Lundborg disease (onset after the age of 6 years) and Lafora disease.

Lafora disease is a distinct disorder with uniform course: onset in teenage years, followed by progressively worsening myoclonus, seizures, visual hallucinations and cognitive decline, leading to a vegetative state in status myoclonicus and death within 10 years.

Biopsy reveals Lafora bodies, which are pathognomonic and not seen with any other progressive myoclonus epilepsies. Lafora bodies are aggregates of polyglucosans, poorly constructed glycogen molecules with inordinately long strands that render them insoluble.

Lafora disease is caused by mutations in the EPM2A or EPM2B genes, encoding the laforin phosphatase and the malin ubiquitin ligase, respectively, two cytoplasmically active enzymes that regulate glycogen construction, ensuring symmetric expansion into a spherical shape, essential to its solubility.

https://pubmed.ncbi.nlm.nih.gov/22961547/

Can diet help with Lafora Disease?

Because Lafora dogs cannot metabolise starch into sugar, it makes sense to keep starch in the diet to a minimum.

As the symptoms progress, you may find that your dog is less able to exercise and will therefore have a tendency to put on weight.

The following general principals may help you decide how best to feed your dog:

1. Avoid any complete food with a high grain content (wheat, oats, rice etc). That includes even the most popular ‘high quality’ foods recommended by vets – some contain up to 50% grain! Check the ingredients carefully.
2. Don’t feed grain based snacks (e.g. Bonios, biscuits etc.) – try green beans, lightly cooked carrots etc.
3. Look into foods that are naturally high in anti-oxidants, such as fish etc.
4. Consider raw feeding – there are convenient, pre-prepared frozen options available that are low in starch, high in protein.
5. Consider home cooked food. Dr Jean Dodds is a US based vet who has developed a home cooked recipe designed especially for epileptic dogs on medications that may adversely affect their liver.

Dr Clare Rusbridge, the UK’s leading expert in Lafora says:

Anecdotally there is some evidence to suggest that dogs with Lafora’s disease are improved on a low GI diet – in other words a diet which is low in simple carbohydrates. Simple carbohydrates e.g. glucose and starch are easily and quickly digested and release glucose rapidly into the bloodstream.

There are many proprietary dog food diets that that have a low GI-index and/or are low in carbohydrates (look for a low grain content) and some owners use homemade diets however, remember that before changing your dog’s diet you should seek the advice of your veterinary surgeon. Starchy/sugary treats may aggravate Lafora’s disease and should be avoided.

http://www.veterinary-neurologist.co.uk/Laforas_disease/

Feed your dog for optimum health

We recommend the following book by Dr Jean Dodds, the top US canine Nutritionist

Breeding

No Miniature Wire Dachshund should be bred unless the owner knows its Lafora status and uses that knowledge to choose a safe breeding combination.

Based on advice from leading veterinary experts, the DBC and WHDC recommend that no Lafora affected dogs should be bred from because:

• Whelping may bring on symptoms early
• Breeding from affected dogs will increase the number of carrier dogs and therefore risk of producing further affected dogs
• Owners accepting the WHDC subsidised test have agreed not to use affected dogs

The Kennel Club records the status of all dogs tested via the WHDC scheme. If you know a dog’s KC Registered Name you can confirm whether not only whether or not it has been tested but what the results were.