WOLMANS DISEASE - ULTRASTRUCTURAL EVIDENCE OF LIPID-ACCUMULATION IN CENTRAL AND PERIPHERAL NERVOUS-SYSTEM

We report the first case of Wolman's disease in which the fine structure of either the peripheral or the central nervous system has been examined. We confirm ultrastructurally the presence of lipid within endothelial and pericytic cells. Several cell types previously believed to be uninvolved in this storage process demonstrate lipid inclusions characteristic of Wolman's disease: perineurial, endoneurial and Schwann cells of peripheral nerve, and oligodendrocytes and astrocytes of the central nervous system. © 1979 Springer-Verlag.