The medical condition known as Globoid Cell Leukodystrophy is also more commonly known as Krabbe's Disease. It is actually a storage disease in which the body of the dog incorrectly processes and stores the enzyme galactocerebrosidase, which is an important component in producing a chemical known as galactocerebroside. Galactocerebroside in turn is essential in the production of myelin that works in the body to coat or protect the nerve endings in the brain and spinal column. When there is not enough myelin produced to provide protection the nerves fire randomly, resulting in staggering walk, tremors and general weakness and lack of coordination in the body.
The breeds of dogs most typically seen with Glaboid Cell Leukodystrophy include Cairn Terriers and West Highland White Terriers. Occasionally the disease can also be seen in Beagles, Basset Hounds, Pomeranians, Miniature Poodles and the Blue Tick Hound. The disease is rare in even the breeds that are associated with the condition and reputable breeders work very hard to identify any carriers of the disease and remove them from the breeding program. Krabbe's Disease is known to be caused by an autosomal recessive gene, which means that both parents must be carriers of the genetic condition for the puppies to have the disease. Since the disease is always fatal if the symptoms are noted, only recessive carriers can pass on the problem gene. Even with two recessive carriers there is still the possibility that not all litters or puppies or not all puppies in a litter will have the condition, but they may be carriers.
Depending on the breed of dog the onset of the symptoms of Krabbe's Disease will become obvious at different points in their life. With the Terrier breeds the condition is usually noted between one and six months of age, but in some of the larger breeds affected the condition may not be noted until after the six month stage. Basset Hounds and Blue Tick Hounds have been reported to not show any symptoms until they are over 1 year old, with onset of symptoms most common between 1.5 and 5 years of age. All puppies will be born completely normal and only after they reach the one month mark will the characteristics possibly develop. Owners should watch for signs of tail tremors, poor growth and development, uncharacteristic lack of coordination that is constant, wide stance, hind end paralysis and vision problems. As the disease progresses vision and even temperament may change as the puppy responds to a decreasing ability to move about and see his or her environment.
As noted earlier there is no cure for Globoid Cell Leukodystrophy and most owners will elect to have the affected puppies put down when the disease interferes with the puppies ability to have a good quality life. There is now a blood test for GCL or Krabbe's Disease for both Cairn Terriers and West Highland White Terriers. Researchers are working on developing a genetic repair for humans with the condition, hopefully this research will soon be available to help the dog breeds that have the genetic factors that contribute to the condition.
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