Neonatal Cerebellar Cortical Degeneration


This is a degenerative disease of the cerebellum, which is part of the brain involved with movement and motor control of the body. This gene appears to only affect beagles in the canine world although there are similar genetic faults in other species. The gene coding for this mutation was sequenced at the Animal Health Trust in the UK.

This was made possible by one of the UK breeders Jaqui Walton who had an affected litter and wanted to know more about the cause. She very bravely donated one of the affected puppies to the AHT and they looked at the family and worked out what was happening.

Otherwise known as NCCD this disease is a genetic disease affecting the beagle, which results in neurological signs very early on in a puppy’s life as soon as they start walking. The disease is not treatable and although there are some beagles, which are able to cope many, are euthanized, as they are unable to walk normally. Affected puppies will lean and fall over as they walk and breeders opt to euthanize them rather than let them reach adulthood. It is a sad disease as in other ways puppies are normal.

It is possible this disease is the same as that described in the USA as “Tumbling Puppy Syndrome” and results from a problem with the production of Purkinje cells which are one of the main nerve cells in that part of the brain. There is also a disease known as cerebellar hypoplasia (CHO) where affected dogs are born with an abnormally underdeveloped cerebellum. This is not the same disease and it is possible in the past the two have been confused. CHO can affect many different breeds.

NCCD is inherited as a simple recessive in the same way as MLS and affected beagles have to carry two copies of the mutant gene to show the disease. It is important to test beagles before breeding, as there are known to be cases of it occurring in the UK. It is safe to breed a carrier to a clear dog, as the resultant puppies would be clear and carriers only. However, if two carriers are bred together 25% of puppies could carry two copies of the gene and thus be affected by the disease.