Bandera's Neonatal Ataxia

Bandera's neonatal ataxia is a serious disease that affects the canine nervous system, specifically the cerebellum, producing tremors, ataxia and nystagmus.

Symptoms

In neonatal Bandera Ataxia, affected puppies have ataxia, tremors and nystagmus (rapid eye movements when the head is immobile) that usually appear in the first weeks of life. Most puppies are unable to walk, although they can move with difficulty.

Disease Management

Given the severity of the symptoms, puppies are usually euthanized or may live a short time with special care. In case your dog shows any symptoms, you should visit your veterinarian for evaluation.

Genetic basis

This disease follows an autosomal recessive mode of inheritance. Autosomal recessive inheritance means that the dog, regardless of sex, must receive two copies of the mutation or pathogenic variant to be at risk of developing the disease. Both parents of an affected dog must carry at least one copy of the mutation. Animals with only one copy of the mutation are not at increased risk of developing the disease, but may pass the mutation on to future generations. Breeding between dogs carrying genetic variants that can cause disease, even if they do not show symptoms, is not recommended.

Technical report

Bandera's neonatal ataxia is named after the first Coton de Tuléar dog in which the pathology was identified. This disease causes alterations in the synapses of the cerebellum and an increase in the number of lamellar bodies of Purkinje cells. The affected gene is GRM1 which codes for the metabotropic glutamate receptor 1. Thanks to the study of Zeng et al. it is known that Bandera's ataxia associated with GRM1 is possibly due to the 62 base pair insertion in exon 8 of GRM1 caused by a retrotransposon.

Most affected breeds

Coton de Tulear

Bibliography

Coates JR, O'Brien DP, Kline KL,et al. Neonatal cerebellar ataxia in Coton de Tulear dogs. J Vet Intern Med. 2002 Nov-Dec;16(6):680-9.

Zeng R, Farias FH, Johnson GS,et al. 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.