Globoid cell leukodystrophy (Krabbe-disease)

Globoidcell leucodystrophy is a lipid storage disorder with progressive degeneration of the white substance of the central nervous system (CNS). The disease is caused by a genetically caused lack of the enzyme galactocerebrosid betagalactosidase. This enzyme is responsible for the lysosomal metabolism of certain galactolipids. Due to the lack of enzyme, these lipids are deposited in the CNS particularly in the multi-nucleated giant cells (globoidcells). The globoidcell leucodystrophy manifests itself in affected dogs at the age of 1-3 months, beginning with ataxia and paresis of the hind legs. During the progress of the disease, muscular atrophy and neurological degeneration oocur. Due to the lack treatment possibilities, the affected animals are usually euthanased after 10 months at the latest.