Figure 6.

Three-year-old child with Krabbe disease and frequent seizures. Deficiency of lysosomal galactocerebroside β-galactosidase (GALC) results in accumulation of toxic psychosine. “Globoid” cells, macrophages containing galactocerebrosides, can be found in enlarged optic nerves. (a) Axial and (b) coronal T2WI show cerebral volume loss with ex vacuo ventriculomegaly, a widespread leukodystrophy with increased white matter signal sparing the U-fibers and corpus callosum, and thickening of the optic chiasm (arrows, b).