Figure 14-64.

Lipid Storage Disease, Globoid Cell Leukodystrophy, Brain, Transverse Section at the Level of the Mammillary Body, Dog.

The white matter, especially of the gyri, has an off-white to light gray appearance (arrows). Macrophages (globoid cells) derived from blood monocytes (also enzymatically deficient in β-galactocerebrosidase) accumulate in white matter to phagocytose galactocerebroside and oligodendroglial debris secondary to the toxic effects of galactosylsphingosine (psychosine) on oligodendroglia (and in the peripheral nervous system Schwann cells). There is also bilateral hydrocephalus of the lateral ventricles, presumably hydrocephalus ex vacuo, as a result of the loss of neurons and their axons.

(Courtesy Dr. H.B. Gelberg, College of Veterinary Medicine, Oregon State University.)