Krabbe's disease, or globoid-cell leukodystrophy, is an inbornerror of lipid metabolism first described in 1916 in two childrenwith spasticity who died in infancy and were found to have "diffusesclerosis" of the brain.1 Prominent features of the brain inaffected children were decreased white-matter mass with relativelynormal gray matter, generalized demyelination, and clustersof globoid cells in the white matter. Subsequent histopathologicalanalyses have demonstrated that the changes in the white matterare due to the death of the myelin-forming oligodendrocytesin the central nervous system and Schwann cells in the peripheralnervous system, whereas the characteristic . . . [Full Text of this Article]
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From the Division of Research Immunology and Bone Marrow Transplantation, Department of Pediatrics, Keck School of Medicine, University of Southern California, and the Children's Hospital of Los Angeles — both in Los Angeles.
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