GALACTOSYLCERAMIDE LIPIDOSIS (KRABBES 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 galac tosylceramide lipidosis, were examined with the scanning and transmiss ion electron microscopes. The diagnosis was established on the basis o f galactosylceramidase activity (0.04 mu katal/kg proteins Versus 0.86 +/- 0.49 in the normal situation) and abnormalities in the white matt er and trouble in myelinization assessed by magnetic resonance imaging . In spite of the fact that enamel, dentin and pulp are extraneural ti ssues, they were also affected by the disease. Ultrastructural observa tions 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 w ere seen. Lysosomal storage inclusions were observed in all the cells of the dental pub. The myelin sheaths of dental peripheral nerves disp layed severe degenerative changes. Proliferation of dentino-clasts con tributed to stimulate root resorption.