Abstract
In canine form of globoid cell leukodystrophy, a considerable degree of myelination was noticed in the CNS of younger dogs. When the disease process advanced, the number of myelinated fibers and oligodendroglia decreased rapidly and many globoid cells appeared in the lesions. Electron- microscopically, the first notable changes were observed in oligodendroglia. They presented various intracytoplasmic inclusions including a formation of membranous “scrolls” in addition to a presence of myelin figures and dense bodies, an accumulation of microtubules and a loss of polyribosomes, prior to and along with myelin disintegration. Furthermore, their cytoplasm often contained polygonal crystalline tubular inclusions which were common features in globoid cells. After these changes, oligoderldroglia degenerated and disappeared. A chronological change of lesions was presented and the pathogenesis of these alterations was discussed.
