Galactosylceramide lipidosis (Krabbe's disease) and deciduous dental tissues. A case report.

The deciduous teeth of a 5-year-old girl affected by a protracted type of infantile globoid-cell leukodystrophy or Krabbe's disease, a galactosylceramide lipidosis, were examined with the scanning and transmission electron microscopes. The diagnosis was established on the basis of galactosylceramidase activity (0.04 mu katal/kg proteins versus 0.86 +/- 0.49 in the normal situation) and abnormalities in the white matter and trouble in myelinization assessed by magnetic resonance imaging. In spite of the fact that enamel, dentin and pulp are extraneural tissues, they were also affected by the disease. Ultrastructural observations of dental mineralized tissues revealed enamel hypoplasia as well as abnormal proteinaceous lamina and hypomineralized lines or bands. Inside the mantle dentin, dendritic inclusions of amorphous material were seen. Lysosomal storage inclusions were observed in all the cells of the dental pulp. The myelin sheaths of dental peripheral nerves displayed severe degenerative changes. Proliferation of dentinoclasts contributed to stimulate root resorption.