by Christine Bennett

Created on: July 26, 2011   Last Updated: July 27, 2011

Krabbe disease is otherwise known as Globoid cell leukodystrophy, but has been called Krabbe disease after Knud Haraldsen Krabbe (1885-1965) who was a Danish Neurologist, exceptionally gifted and publishing his first scientific paper at the age of only 10. He became a professor of neurology and chief of neurological service in Copenhagen, and he continued to produce papers even when suffering from Parkinsonism until his death at 80 yrs of age.

First diagnosed in 1963 in West Highland Whites, it is a rare but often fatal disorder that is degenerative and affects the myelin sheath of the nervous system. It is found in humans and mice as well as dogs, and in children it is often fatal before the age of 2 years.

A dog can be clear of the disease, infected or a carrier as this is an inherited autosomal recessive trait, a carrier can actually pass the disease to 50% of its offspring without being affected itself.

Dogs mostly inflicted with this condition are West Highland Whites and Cairn Terriers but it has been seen in Beagles, Miniature Poodles, Blue Tick Hounds, Pomeranians and Basset Hounds.

Recently it has also been discovered in Australian Working Kelpies when in 1998-1999 three of these dogs from the same litter were found to have Krabbes disease.

In Krabbes disease it is the lack of an enzyme called B-galactocerebrosidase which results in too much galactocerebroside, a component of myelin, which then causes disruption of the cells which produce the myelin. 

Myelin is a fatty substance which coats the nerve cells and acts as an electrical insulator, crucial for the nerve impulses to remain normal. If the loss of myelin continues then the nervous system which consists of the brain, spinal cord and peripheral nerves will all be affected causing various signs in the dogs.

When the myelin decreases and the nervous system is affected, the signs appearing in the dog will include lack of co-ordination, tremors, weakness and loss of balance.

Puppies will be born with the appearance of being quite normal, but the symptoms will start to appear around 1-3 months afterwards with weakness in the limbs being the first symptom, followed by tremors and muscular atrophy with neurological deterioration.

Puppies tend to show a weakness in their hind limbs by first appearing stiff or perhaps falling to one side, often a change in behaviour is noted and sometimes vision is affected too.

Dogs have lived to 8-9 months but are usually euthanized as there is no cure for this

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