SCA and LOA in Parson Russell Terrier, Jack Russell Terrier and other breeds

Hereditary ataxias are congenital disorders of the central nervous system in the dog breeds Fox Terrier, Parson Russell Terrier and Jack Russell Terrier. Characteristic clinical signs include uncoordinated and exaggerated movements, loss of hearing, collapse and falling over sideways, associated with the inability to stand up again on one's own. In this disease there is a loss of the white matter of the nerve fibers in the spinal cord and cerebellum (neuronal axonopathy). Different forms are distinguished.

Cerebellar Ataxia (CA) occurs in puppies as early as 2 weeks of age when they begin their first attempts to walk and results in changes in the cerebellum. Spinocerebellar ataxia (SCA) occurs between 2 and 6 months of age. Late manifest ataxia (LOA), on the other hand, occurs at 6 to 12 months of age. In SCA and LOA, the spinal cord and brain are affected and affected dogs show clinically very similar characteristic ataxia symptoms.

Hereditary ataxias are inherited in an autosomal recessive manner. However, it is likely that different mutations can cause hereditary ataxias and thus a monogenic inheritance is not sufficient for the clinically diagnosed hereditary ataxia cases. Mutation tests are available for SCA and LOA, which have been previously demonstrated in the Parson Russell Terrier and Jack Russell Terrier breeds. The mutation considered responsible for LOA has been validated in Parson Russell Terriers and Jack Russell Terriers. SCA is associated with a mutation in the gene KCNJ10 and LOA is associated with a mutation in the gene CAPN1.

For the mutation tests for SCA and LOA in Parson Russell Terriers and Jack Russell Terriers, the following test results are possible:

1. Plant-free:
The dog has no predispositions for SCA or LOA. These animals do not inherit any predispositions for SCA or LOA.

2. Plant carrier:

The dog cannot have SCA or LOA, but can pass a copy of the predisposition to 50% of its offspring. When mated to a trait carrier, there is a 25% chance of having diseased puppies.

3. Trait carrier:
The dog will have a high probability of developing SCA or LOA.

If the test results for SCA or LOA do not agree with clinical observations and/or veterinary findings, please be sure to contact us. In the case of mutation tests, it cannot be ruled out that very similar disease patterns are caused by mutations not yet known. According to the investigations carried out so far, it is possible that in the case of the hereditary ataxias of the Parson Russell Terrier and Jack Russell Terrier, further mutations may lead to a very similar course of the disease.

The performance of mutation tests for SCA and LOA in Parson Russell Terriers and Jack Russell Terriers is subject to a fee (submission form).

Hereditary ataxia with degenerative changes in the brain and spinal cord have also been described in other breeds such as Dachshund and Epagneul Breton. For the research of Hereditary Ataxia in other breeds, we request the submission of blood samples from the following breeds. Support for further research of Hereditary Ataxia in the breeds listed below is free of charge (submission form).

  •     Fox Terrier
  •     Dachshund
  •     Epagneul Breton