Working together toward a better breed.
The Jack Russell Terrier Research Foundation
Ataxia
In January 2004, the Foundation Board approved additional funding for the ataxia research project, headed by Dr. Paige Carmichael, at the University of Georgia. The project was funded by a $3,000 donation from the GA Jack Russell Terrier Club with matching funds of $3,000 from the Foundation. Special appreciation is due the Georgia Club for their relentless support and contributions in an effort to understand and prevent this fatal disorder.
Please take a moment view these
two videos of a puppy with Ataxia.
We thank the owner of this puppy for donating these videos.
Frequently Asked Questions About Ataxia
Cerebellar Ataxia
By Dr. Paige Carmichael
Cerebellar ataxia is a non-specific term referring to lack of balance (ataxia) that can be traced to the part of the brain called the cerebellum. There are many causes of cerebellar ataxia in dogs, including toxins, trauma and infectious agents. Inherited cerebellar ataxia is a specific form of ataxia in Jack Russell Terriers. It is a progressive brain disease caused by premature death of a particular brain cell called the granule cell. Granule cells are found in the cerebellum, the part of the brain that is responsible for balance. This inherited disorder has been recognized in the JRT in the United States for several years.
In the form of ataxia studied by researchers at the University of Georgia, the disorder is inherited as an autosomal recessive condition. Studies have shown that both parents must be carriers to produce an affected litter. In affected litters, puppies can show signs as early as two to four weeks of age right after they start walking. Males and females are equally affected. They show head bobbing, a wide-based stance (tripod stance) and knuckling over at the paws. This will progress to stumbling and falling over, and in some cases, inability to stand. UGA researchers have observed that there is some variability in the clinical signs that dogs may show. In the same litter, there may be dogs that are recumbent and unable to rise, while others are able to walk with help.
In the past few years, ataxic dogs have been located in many states as well as Canada and Europe, emphasizing the widespread nature of this disorder. The researchers at UGA are examining certain microsatellite markers in affected and normal JRTs. When a mutation is identified in an affected dog that is not present in its normal counterpart, a test for the mutation will be developed and used in initial screening studies. These genetic abnormalities found will allow them to develop tests that will allow breeders to make informed breeding decisions.
Q: What is the name of the disease?
A: Inherited Cerebellar Ataxia.
Q: What causes Inherited Cerebellar Ataxia?
A: This disease is caused by inherited cell loss in an important part of the brain called the cerebellum.
Q: What does the cerebellum do?
A: The cerebellum is the part of the brain that controls balance.
Q: Does the disease affect any other part of the brain?
A: No. The dogs with Cerebellar Ataxia are mentally normal.
Q: When do you see the first symptoms?
A: The signs appear when the pups are between 2 and 4 weeks old.
Q: What are the first symptoms?
A: Shaking in general. Head bobbing, difficulty in standing, goose-step like motion, falling down from side to side, splayed out front legs when eating and drinking as if they are trying to stabilize themselves.
Q: Can there be variation in the severity of the symptoms?
A: Yes, some dogs can still walk, while others cannot rise at all.
Q: Are dogs with Cerebellar Ataxia going to die?
A: Cerebellar Ataxia puppies can live for many years with intense supportive care.
Q: Will puppies with Cerebellar Ataxia get worse?
A: Yes, the disease is progressive.
Q: How much worse will they get?
A: The severity varies. Some dogs will not be able to stand by the time they are seven weeks old, while other dogs can walk with difficulty indefinitely.
Q: Are there any other breeds of dogs that have this problem?
A: Many breeds of dogs have similar forms of cerebellar ataxia.
Q: Is this syndrome like any other in JRTs?
A: There is another syndrome that is called Spinocerebellar Ataxia. That has a later onset of about 5-8 months of age and is not as debilitating to the dog.
Q: Are Cerebellar Ataxia and Spinocerebellar (late-onset) Ataxia related?
A: No, test matings have shown that these two forms are not related.
Q: How widespread is Cerebellar Ataxia?
A: Cases have been reported throughout the country.
Q: What causes Cerebellar Ataxia?
A: It is a genetically inherited trait.
Q: What is the inheritance pattern?
A: It is a recessive trait. That means that both parents have to be carriers of the trait (and be normal) to produce the sick puppies.
Q: What are the chances of getting an affected dog if two carriers are mated?
A: There is a 25% chance the puppy will be affected, a 50% chance that the dog will be carrier of the disease but be normal, and a 25% chance that the dog will not be a carrier of the disease and will be normal.
Q: What is the purpose of Dr. Carmichael's research?
A: The purpose of the research is to understand the cause of the disorder and to find a test that can detect carriers.
Q: Why do we need a genetic test?
A: We need the genetic test to be able to identify carriers of the disorder in the general population.
Q: How long is it until we have a test?
A: We have no way of knowing, but we are working as fast and as hard as we can.
Q: How much will the test cost?
A: We don't know.
Q: When the test is ready, what will we need to do the test?
A: We plan to be able to do the test using no more than 3mls of EDTA-preserved whole blood.
Q: What can we do to help?
A: We always need money to continue the research project. However, Dr. Carmichael is looking for general pedigree information and blood samples. We also need normal tissue samples.
Q: What happens to any information that is provided to Dr. Carmichael?
A: All information provided will be kept totally confidential and will only be seen and used by the personnel working in the lab.
Q: What can I do if I have a JRT ataxic litter?
A: Contact Dr. Carmichael (kpc@vet.uga.edu) or the technicians (cunger@vet.uga.edu, ozenh@uga.edu) in her lab at the University of Georgia.
Q: Can I donate the ataxic puppies?
A: Yes, and please be assured that they will be well cared for and loved.
All donations will be greatly appreciated. If you have any other questions or comments please let us know. You can contact us at the above emails or write us at the above address
Dr. Paige Carmichael
College of Veterinary Medicine
University of Georgia
Athens, GA 30602
(706)542-5834 (6373)
Researchers
Dr Joan Coates
University of Missouri
Dr. Paige Carmichael, DVM
University of Georgia
Hope
Hope's Story
Hope was born with Cerebella Ataxia. Cerebella Ataxia is a defect in the part of the brain that controls balance. Hope seemed normal to us at birth, however, by the time she was between 2-3 weeks old, it was apparent that there was a problem. Hope just laid in the whelping box while her siblings got up and played all over her. A neurologist confirmed our fears, but we decided to keep Hope and give her the best life possible. While she does not walk like a normal dog, she is able to play with our other terriers and has brought us much joy. Hope will be 5 years old this July.
Darlene McInnes