Lafora’s Disease in MWHD
Lafora’s disease is an inherited, late onset, progressive myoclonic epilepsy. This degenerative neurological disease has been identified in Miniature Wirehaired Dachshunds. The disease is characterised by myoconus. Typically this looks like a backwards shuddering/jerking of the head when there is movement towards the eyes, when light intensity increases, when there is flickering light (e.g. television) or at sudden noises. Some dogs also develop epilepsy. Middle aged to older dogs (age range 5-8 years) of both sexes can be affected. Unfortunately there is not a completely effective treatment, however many are improved on anti-epileptic drugs.
Although the myoclonus (jerking) and epilepsy can get worse it does not appear to shorten the life of affected dogs. The disease progresses slowly over many years and gradually other neurological deficits such as ataxia, blindness and dementia occur. Because affected dogs are missing a vital enzyme involved with carbohydrate metabolism this results in the storage of a polyglucasan storage material (Lafora bodies) within the brain and some other tissues. The material interferes with synaptic transmission.
The abnormal gene which causes this disease has been identified and a DNA test is available (but only from a laboratory in Canada). This is especially important as the disease develops after the normal breeding age, so an early test could provide a way for breeders to “breed away” from this problem.
The genetic mutation in the Dachshund was identified at The Hospital for Sick Children, Toronto. The Hospital offers a test for affected dogs in return for a $200 (Canadian dollars) donation to the hospital (credit cards are accepted). Tel: 416-813-7721 or e-mail: email@example.com (Berge Minassian – profile)
Download the Breed Council’s Lafora Leaflet for details of Lafora screening available in the UK.
Read Elsa Beltran’s (AHT) presentation on Lafora Disease (Feb.2011)
BBC News article on Lafora’s (2005)
Our Dogs article (2005)