Neuronal Ceroid Lipofuscinosis (NCL), also known as Cerebellar Cortical Degeneration, is a disease with progressive loss of neurons within the cerebellum, and subsequently in other regions of the brain. This results 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 varies but is usually between three and six years of age. The rate of progression also varies between dogs, progressing over months to years.

A genetic mutation (ARSG) has been identified in association with the development of NCL in American Staffordshire Terriers as well as Staffordshire mixes. This is a recessive condition, so dogs that have only one copy of the gene do not develop clinical signs. If a dog is negative or heterozygous for this mutation but has signs of cerebellar disease, it is likely due to another cause.

Testing Prices:

American Staffordshire Terrier NCL = $48.00 per dog

American Staffordshire Terrier Litter Discount = $38.00 per puppy

Sample Type for Submission:

Acceptable samples include a cheek swab or 1-2 mL whole blood collected in an EDTA tube. If these sample types are unavailable, please reach out to us at [email protected] for more information. Alternative sample types may result in an additional charge.

Explanation of Results:

Two copies of each gene are inherited, one from each parent. Possible results include:

Negative

Dogs have two copies of the normal gene, which is not associated with the development of NCL. They cannot transmit this mutation to their offspring.

Positive Heterozygous

Dogs have one copy of the normal gene and one copy of the mutated gene. Dogs will not show clinical signs as this is a recessive disorder. However, dogs may be able to transmit a mutation copy of the gene to their offspring.

Positive Homozygous

Dogs have two copies of the mutated gene, which is associated with NCL and the development of clinical signs. They will transmit one copy of the mutation to their offspring.

An estimated 98% of dogs positive homozygous for this mutation have developed clinical signs of NCL. Other factors could contribute to this condition in dogs, and a negative result does not rule out the presence of a different mutation that could cause a similar genetic disorder or trait.

Breeding Recommendations:

Breeding decisions should be made carefully and in discussion with your veterinarian. We cannot give specific breeding recommendations regarding your dog, but we advise to consider these genetic results as well as the overall health of the line. The goal is to reduce the prevalence of these disease-associated mutations while maintaining high quality variety within the population.