Gangliosidosis (Storage Disease) in Dogs

Gangliosidosis is a recessive, genetic disorder. Screening for the gene is not commonly done in all breeds. Breeds most commonly affected by storage diseases are Japanese Chins, Beagle crosses, German Shorthaired Pointers, Portuguese Water Dogs, and the Springer Spaniel. It would be fair to ask a breeder from whom you are considering purchasing a puppy, if both parents have had the DNA test for gangliosidosis, especially of those breeds more commonly affected.

Gangliosidosis is a rare metabolic disorder in dogs. It is commonly known as storage disease because dogs that suffer from it lack an enzyme in their brain that  helps with the breakdown of old molecules. Without the ability to rid the brain of old molecules as new ones grow, the result is a build up in the brain that affects the nervous system.

Symptoms can vary greatly between cases but could include:

• Lack of coordination
• Depression
• Behavioral changes
• Head shaking (especially  when eating)
• Mental dullness
• Seizures
• Blindness
• Deafness
• Developmental delay
• Unaware of surroundings

Types

Gangliosidosis is a type of metabolic disorder. Dogs and humans can suffer from disorders related to the production or the breakdown of molecules in the brain or elsewhere. Other storage diseases include:

• Ceroid lipofuscinosis
• Krabbe disease
• Sphimgomyelinosis
• Batten disease
• Gaucher disease
• Mucopolysaccharidosis

Gangliosidosis is a rare, recessive genetic disorder. Dogs carrying the recessive gene should not be bred with another dog carrying the recessive gene or arguably, not at all. Siblings of dogs carrying the gene mutation should certainly be screened before being entered into a breeding program. It is also possible for dogs to eat a poisonous plant that interrupts the enzyme process and acquire a storage disorder that way. Genetics are still the most probable cause.

Symptoms of gangliosidosis in dogs are hard to recognize. Because the disorder comes from the build up of old molecules in the brain the dog may appear normal for some time. Gangliosidosis is a relatively rare affliction, so it may not be on the forefront of your veterinarian's diagnostic mind. When build up becomes severe, the dog’s coordination is affected. Your dog may seem lethargic or depressed. Often head shaking is observed, especially when eating. Your dog may experience seizures. Canine distemper or liver toxicity may resemble symptoms of gangliosidosis. Because these are more common problems, your veterinarian may first mistakenly diagnose your dog. The symptoms are vague and resemble other afflictions. Once gangliosidosis is suspected, your veterinarian can confirm diagnosis with a blood test.

There is no specific treatment for gangliosidosis in dogs. Unfortunately the dog’s quality of life diminishes until euthanasia is considered the most humane choice. Because human’s are affected by similar disorders gene therapies are being tested that may help in the future.

Gangliosidosis is considered fatal in dogs. There are no options for recovery. Prevention through DNA testing and good breeding practices are the best way to avoid this heartbreaking affliction. Euthanasia usually happens within six months of diagnosis. The dog is most commonly an adolescent. This is just enough time for the entire family to be entirely attached to the dog and the loss is tragic. Many breeders will sell puppies with a “health guarantee.” As distressing as it may be, a case of storage disease would be an appropriate time to press your breeder on that guarantee. Most guarantees require the return of the puppy. Breeders are aware that most people will not return a dog they have grown attached to. In a case where euthanasia is the only option and the loss of the dog is unavoidable, you can make the breeder aware of the recessive gene the bitch and stud are carrying.