The peripheral neuropathy of canine globoid-cell leukodystrophy (Krabbe-type)

A variety of peripheral nerves were studied by light microscopy from 7 young Cairn Terrier dogs which were necropsied and histologically diagnosed as having canine globoid-cell leukodystrophy (Krabbe's type). All nerves examined had severe degenerative changes in axons and myelin sheaths, similar to lesions in human cases of Krabbe's disease. Endoneurial fibrosis was not prominent but many PAS positive macrophages and a mode-rate number of mast cells were in the endoneurium. However, the dorsal root ganglia had no detectable changes in neurons nor proliferation of capsule cells or interstitial cells. Myopathy in the form of muscle atrophy and degeneration of muscle nuclei was seen in two dogs. Thus, in canine globoid-cell leukodystrophy, lesions occur widespread in the peripheral as well as in the central nervous system.