Neonatal encephalopathy with seizures is an inherited progressive brain disease of Standard Poodles and related crosses that is characterized by weakness, mobility issues, and seizures. Affected pups do not survive beyond seven weeks of age.
Phenotype: Newborn pups affected by neonatal encephalopathy with seizures (NEWS) typically appear significantly weaker than littermates. If they survive the first week, mobility issues and seizures may be observed. Affected pups do not survive beyond seven weeks of age.
Mode of Inheritance: Autosomal recessive
Alleles: N = Normal, ES = Neonatal encephalopathy with seizures
Breeds appropriate for testing: Standard Poodle, Labradoodle, Goldendoodle, other Standard Poodle crosses and lineages
Explanation of Results:
Dogs with N/N genotype will not have neonatal encephalopathy with seizures and cannot transmit this variant to their offspring.
Dogs with N/ES genotype will not have neonatal encephalopathy with seizures, but are carriers. They will transmit this variant to 50% of their offspring. Matings between two carriers are predicted to produce 25% of puppies affected by neonatal encephalopathy with seizures.
Dogs with ES/ES genotype will have neonatal encephalopathy with seizures, a fatal condition.
Neonatal encephalopathy with seizures (NEWS) is an inherited progressive brain disease of Standard Poodles caused by a mutation in the activating transcription factor 2 (ATF2) gene. NEWS is inherited in an autosomal recessive fashion thus both sexes are equally affected. Two copies of the defective gene must be present to cause the disease. Effects of the disease can be observed in newborn pups which typically appear significantly weaker than littermates. If they survive the first week, mobility issues and seizures may be observed. Affected pups do not survive beyond seven weeks of age. The NEWS mutation is widespread across Standard Poodle lineages and colors. Dog breeds developed with Standard Poodle breeding stock are at risk of NEWS. Dogs with one normal and one mutated ATF2 gene (carriers) are unaffected but breeding two carriers together would be predicted to produce 25% affected offspring and 50% carriers.
Testing for NEWS assists owners and breeders in identifying affected and carrier dogs. Breeders can use results from the test as a tool for selection of mating pairs to avoid producing affected dogs.
Carrier. 1 copy of the NEWS mutation. Dog is normal.
Affected. 2 copies of the NEWS mutation. Dog is affected.
Chen, X., Johnson, G.S., Schnabel, R.D., Taylor, J.F., Johnson, G.C., Parker, H.G., Patterson, E.E., Katz, M.L., Awano, T., Khan, S., O'Brien, D.P. (2008). A neonatal encephalopathy with seizures in standard poodle dogs with a missense mutation in the canine ortholog of ATF2. Neurogenetics, 9(1), 41-49. doi: 10.1007/s10048-007-0112-2