Back

Neonatal Ataxia

Neonatal Ataxia (NA), or Bandera's Syndrome (BNAt), is an early onset inherited neurological disorder in Coton de Tulears, affecting coordination and movement due to improper cerebellum function.

Affected Genes: GRM1

Inheritance: Autosomal Recessive

Variant(canFam6):
chr1:38456676-38456677: 62 bp insertion AAGACCCGCAACGT(A)48); with 14 bp duplication AAGACCCGCAACGT

Breed: Coton de Tulear

General Information: Neonatal Ataxia (NA), also known as Bandera's Syndrome (BNAt), is an inherited neurological disorder that affects Coton de Tulear dogs shortly after birth. This condition results from improper functioning of the cerebellum, the part of the brain responsible for coordination and movement. Affected puppies exhibit symptoms soon after birth, around the time when their normal littermates start developing coordinated movement. These symptoms include an inability to stand or walk properly, leading to scooting on their stomachs, frequent falls, and leg paddling. Additionally, these puppies often develop tremors. Although the severity of the disease does not progress after four months of age, the poor quality of life typically leads to euthanasia.

How to Read Your Dog's Test Results for this Genetic Variant:

Two Variants Detected: Dog Likely Affected

One Variant Detected: Dog Unlikely Affected

No Variants Detected: No Effect

Gene / Testing Information: Genetic testing for Neonatal Ataxia (NA), also known as Bandera's Syndrome (BNAt), involves screening for mutations in the GRM1 gene to determine carrier status. This disorder is inherited in an autosomal recessive manner, meaning a dog must inherit two copies of the mutated gene, one from each parent, to develop the disease. Carriers of the gene mutation do not exhibit symptoms but can pass the mutation to their offspring. When two carriers are bred, each puppy has a 25% chance of being affected and a 50% chance of being a carrier. To prevent producing affected puppies and eliminate the mutation from breeding lines, it is crucial to avoid breeding two carriers. Dogs that are not carriers of the mutation do not pose a risk of producing affected puppies, making genetic testing an essential tool in responsible breeding practices.

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 16(6):680-689.

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 25(2):267-272.