Skip to main content

Genetics: American Staffordshire Terrier Neuronal Ceroid Lipofuscinosis

This condition is also known as Cerebellar Cortical Degeneration and has been documented in American Staffordshire Terriers as well as mix breed dogs. It is caused by a mutation in the Arylsulfatase gene (ARSG).

American Staffordshire ARSG Mutation Testing Price: $48.00 per dog, or $38.00 per puppy for two or more puppies of the same litter

Progressive loss of neurons within the cerebellum, and subsequently in other regions of the brain result initially in subtle signs of loss of balance, particularly when shaking the head, or making a fast turn. These signs progress to an uncoordinated gait, with high stepping, swaying of the trunk and frequent falls. Onset is usually between 3 and 6 years of age but can be earlier or much later and rate of progression is over months to years and varies between dogs.

This is a recessive condition, and so dogs that have only 1 copy of the gene do not develop clinical signs. Of 142 dogs that were homozygous for the mutation, 4 did not show signs at time of testing, thus 2% of dogs that tested positive did not, to our knowledge, develop signs of disease.

Once you run the DNA test we will send you results that state your dog’s results are either:

  • Negative – This means that your dog does not have the genetic mutation for Cerebellar Cortical Degeneration in American Staffordshire Terriers and cannot pass it on to their puppies.
  • Positive Heterozygous – This means that your dog has 1 copy of the genetic mutation and 1 copy of a normal gene. Since this is a recessive disease dogs with 1 mutation do not develop the clinical signs but is considered a carrier of the trait. If your dog has signs of cerebellar disease, it is likely due to another cause.
    • Breeding recommendations: If the dog has many other positive traits it may be reasonable to consider breeding these dogs to a Negative dog, screening the puppies and trying to select a Negative puppy to keep as a replacement breeding animal in the next litter or so. Over time this will gradually reduce the prevalence of the disease in the breed. We do not recommend breeding a positive heterozygous dog to a positive heterozygous dog since this could produce positive homozygous dogs which will develop this disease.
  • Positive Homozygous – This means that your dog has 2 copies of the genetic mutation and can be diagnosed with this disease if it is showing signs. Breeding recommendations: your dog has a 100% chance of passing the mutation on to all of its puppies. We would not recommend using these dogs for breeding purposes if possible.