Globoid Cell Leukodystrophy
Globoid cell leukodystrophy (GCL, or Krabbe's disease in humans) is a degenerative disease of the brain and spinal cord associated with gradual and symmetrical destruction of the white matter of the brain. It occurs from a lack of myelin, a necessary lipid (fatty substance) that coats the spinal cord and other nerves. It's the result of a genetic deficiency of an enzyme (galactocerebroside beta-galactosidase) involved in the breakdown of certain fats in the brain and spinal cord.
SIGNS begin early in life and progress rapidly and include weakness, stumbling, loss of control of the hindquarters, and tail tremors. Then the dog will develop a wide stance, lack of coordination, and hind or total limb paralysis. He may become blind or fail to recognize familiar individuals. GCL may occur as early as 4 weeks of age and nearly always occurs before 5 to 6 months of age. The typical clinical signs and measurement of the main activity of galactocerebroside beta-galactosidase in white blood cells will confirm a positive diagnosis.
Globoid cell leukodystrophy seems to affect a small number of related Irish setters and Dalmatians. Other dog breeds reported with GCL include Pomeranian, Miniature Poodle, Basset Hound, Beagle, and Blue Tick coonhound. The disease has been seen in domestic shorthaired kittens in which it causes progressive degenration of the nervous system and death between 2 and 6 months of age.
Treatment of Globoid Cell Leukodystrophy No treatment exists; GCL is fatal. Most common in Westies and Cairns, the disease is known to be a an autosomal recessive disease (two parents are required).
Fortunately, an accurate blood test (using DNA technology) is now available to concerned breeders. It can accurately identify a dog as a carrier, as affected, or as clear of the disease. Testing a small blood sample identifies "carriers," whose offspring have a 50% chance of carrying the gene, but who will themselves be clear. Breeders should test any dog in their breeding program to determine whether or not he's a carrier. This important tool is an opportunity to minimize the number of carriers in the Westie population and eventually eliminate GCL altogether. Any bloodline contaminated with the defective gene will produce affected puppies when linebreeding on the source of the defect.
Go Pets America recommends seeking the advice of your local veterinarian for the most appropriate vaccination program and for the diagnosis and treatment of your pet's health problems. For vaccination requirements please contact your state and local licensing authorities.
|