Neonatal Ataxia

Other Names: Bandera's neonatal ataxia, Bandera's syndrome, Neonatal ataxia, Neonatal cerebellar ataxia, BNAt
Affected Genes: GRM1
Inheritance: Autosomal Recessive
Mutation: Complex Rearrangement
Breed(s): Coton de Tulear

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Common Symptoms

Neonatal cerebellar Ataxia is an early onset, inherited neurologic disease affecting Coton de Tulears. Symptoms present shortly after birth when normal littermates develop coordinated movement. In affected dogs the Cerebellum does not function properly and pups do not develop the balance necessary to stand or walk normally. Affected puppies tend to scoot on their stomachs to move and often fall to their side and paddle their legs. These pups usually develop tremors as well. While the disease does not appear to progress in severity after 4 months of age, puppies are usually euthanized due to a poor quality of life.


Testing Tips

Genetic testing of the GRM1 gene will reliably determine whether a dog is a genetic Carrier of neonatal Ataxia. Neonatal ataxia is inherited in an Autosomal Recessive manner in dogs meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease. In general, carrier dogs do not have features of the disease but when bred with another carrier of the same Mutation, there is a risk of having affected pups. Each pup that is born to this pairing has a 25% chance of inheriting the disease and a 50% chance of inheriting one copy and being a carrier of the GRM1 gene mutation. Reliable genetic testing is important for determining breeding practices. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Dogs that are not carriers of the mutation have no increased risk of having affected pups.


There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.


References

  • Coates JR, O'Brien DP, Kline KL, Storts RW, Johnson GC, Shelton GD, Patterson EE, Abbott LC. Neonatal cerebellar ataxia in Coton de Tulear dogs. J Vet Intern Med. 2002 Nov-Dec; 16(6):680-9. [PubMed: 12465765]
  • Zeng R, Farias FH, Johnson GS, McKay SD, Schnabel RD, Decker JE, Taylor JF, Mann CS, Katz ML, Johnson GC, Coates JR, O'Brien DP. A truncated retrotransposon disrupts the GRM1 coding sequence in Coton de Tulear dogs with Bandera's neonatal ataxia. J Vet Intern Med. 2011 Mar-Apr; 25(2):267-72. [PubMed: 21281350]