Dysmyelination of the central nervous system in the Chow-Chow dog

Five Chow-Chow dogs from three different litters had generalized body tremors since birth. Histologically a severe myelin deficiency was found in the central nervous system. The peripheral nerves were normally myelinated. Electron microscopically abnormally thin myelin sheaths and completely naked nerve fibers were found. There were many astrocytes in the myelin deficient areas as well as cells with the characteristic features of oligodendrocytes but also containing fibrils. The present cases are compared to other dysmyelinating diseases and possible pathogenetic mechanisms are discussed.