Brain Cell Degeneration in Dogs

Brain cell degeneration in dogs, known as cerebellar abiotrophy, is an inherited condition in which the cerebellar tissue of the brain degenerates over a period of time. This neurologic disorder can happen in several different dog breeds, especially in puppies. Cerebellar abiotrophy occurs when the nutrients needed for the cells are no longer present. Once the cells of the cerebellum do not receive the nutrients needed, the cerebellum, which is responsible for the dog’s balance and the many movements a dog needs to do, is unable to perform properly. The cerebellum is the vital section of the brain that is responsible for the body’s movements and coordination. In cerebellar abiotrophy, the cells prior to birth are normal; however, begin to deteriorate soon thereafter in some puppies, or too early in some dogs.

Brain cell degeneration in dogs, or cerebellar abiotrophy, is the premature deterioration of cells in the cerebellum region of the brain.

The symptoms of cerebellar abiotrophy are usually those associated with balance and movement. They also do occur at various times, depending on the specific dog breed that is affected. Some signs can occur anywhere from four to sixteen weeks in puppies, and some dog breeds develop symptoms in the very young adult stage of life. Symptoms include:

• Loss of balance
• Falling over
• Shaking the head, as if something is in the ear
• Head tremors
• Difficulty walking
• Difficulty getting up from a resting position

Types

Cerebellar abiotrophy is an inherited condition that is harmful to dogs of a variety of ages, yet can be breed-specific. Common breeds that are more susceptible to developing this disease can include:

• Airedale Terrier
• Australian Kelpie
• Beagle
• Bernese Mountain Dog
• Miniature Poodle
• Miniature Schnauzer
• Border Collie
• Bullmastiff
• English Bulldog
• Irish Setter 
• Jack Russell Terrier
• Labrador Retriever

Since this disease is breed-specific, the causes are limited to these breeds.  The inheritance gene is a recessive gene (autosomal), and this abnormal gene causes an abnormality in glutamate metabolism.

• Genetic predisposition
• Defect in the glutamate metabolism
• Canine herpesvirus in utero (possible)
• Canine herpesvirus as a newborn (possible)

If you see any symptoms, make an appointment with your veterinarian. The veterinarian will ask you questions concerning the dog’s history and review his health file. After hearing the symptoms he is having, he will then perform several tests to rule out any other conditions and to confidently come up with the diagnosis. 

He will perform blood tests, tests for any infectious diseases, and a urine analysis. He will also need to rule out any other similar-type of neurological diseases that may have similar symptoms. He will more than likely perform a CSF tap, screenings for urine acids and check for lysosomal storage diseases, and he will do an MRI. He may be able to test the brain tissue of the dog, as well. These tests are necessary to determine the exact disease; however, the only determining factor for cerebellar abiotrophy is a brain biopsy after the dog has passed.

If the puppy was normal at birth and then develops specific signs to cerebellar abiotrophy right after birth at various time frames, this will give more clues to the veterinarian that he may have cerebral abiotrophy.

Unfortunately, there is no known effective treatment for any dog that suffers from brain cell degeneration, or cerebellar abiotrophy.

If your dog has a mild case, this disease is not always fatal. Even though there is not a cure, he may still be able to live for a certain period of time with your help in keeping him comfortable and happy. Every dog is different, and some are negatively affected more than others. Your dog may have poor coordination, movements, and seem very confused. Be sure to keep your dog safe by keeping him away from any risks that may harm him, such as swimming pools, steps, sharp objects, or anything that he can get into that can hurt him. 

Exercise will need to be restricted and he may need help with getting around. He may also need to be helped with using the bathroom; you may need to be sure he is kept comfortable and cleaned if he gets urine or feces on himself. If he does have a serious case of this disease, he will need to have a lot of care in the above methods. It will be very important to keep your dog comfortable and to give him lots of love during this difficult time. Only you as a loving pet owner will be able to decide when to euthanize your companion.