Gopetsamerica.com

Globoid Cell Leukodystrophy

Globoid cell leukodystrophy (GCL0, known as 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. The disease results from a genetic deficiency of the enzyme galactocerebrosidase (GALC) involved in the breakdown of certain fats in the brain and spinal cord. Its name is derived from the characteristic storage cells found around cerebral blood vessels in the white matter of affected animals. This condition is fatal.

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, West Highland White Terrier, Cairn Terrier, and coonhounds. The disease has also been seen in domestic shorthaired kittens in which it causes progressive degeneration of the nervous system and death between early in life.



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 galactocerebrosidase (GALC) enzyme in white blood cells will confirm a positive diagnosis.

An accurate blood test using DNA technology is now available to concerned breeders. It can effectively 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.

References

  1. Jill Arnel. The West Highland White Terrier
  2. The Official Book of the Dalmatian
  3. Sheldon L. GerstenfeldThe Cat Care Book: All You Need to Know to Keep Your Cat Healthy and Happy
  4. Curtis W. Dewey. A Practical Guide to Canine and Feline Neurology

Related Conditions

Arnold-Chiari Malformation

Progressive Axonopathy

Canine Degenerative Myelopathy

Canine Wobbler Syndrome

Cerebellar Abiotrophy

Cerebellar Hypoplasia

Cerebellar Purkinje cell degeneration

Cranioschisis

Degenerative Myelopathy

Polyneuropathy, Distal Sensorimotor

Dysautonomia

Epilepsy

Encephalomyelitis

Fucosidosis

Gangliosidosis, GM1, GM2

Globoid Cell Leukodystrophy

Horner Syndrome

Hydrocephalus

Hydromyelia

Hypomyelination

Lafora Disease

L-2-Hydroxyglutaric Acidemia

Menkes Syndrome

Motor Neuron Disease

Myasthenia Gravis

Necrotising Myelopathy

Neuronal Ceroid Lipofuscinosis (NCL)

Neuronopathy, progressive

Nervous System Disorders

Peripheral Nerve Conditions

Polyneuropathy

Purkinje Cell Degeneration

Pug Dog Encephalitis

Spina Bifida

Syringomyelia

Veterinary Drugs



HomeDogsCats Birds Fish Horses Reptiles Small Pets Jobs Animals Biology Garden Pics Video Search Contacts

©2015 Go Pets America, Inc. All Rights Reserved.