LEPTOMENINGEAL LIPID STORAGE PATTERNS IN FABRY DISEASE

We found two patterns of leptomeningeal storage that reflect two basic visceral storage patterns in Fabry disease. (i) A generalized-type le ptomeningeal storage pattern, affecting all main leptomeningeal cell t ypes (external arachnoideal epithelium, fibroblasts, vessel wall eleme nts), was a consistent finding in three cases of classical generalized visceral phenotype. (ii) A localized leptomeningeal storage pattern w as expressed, to a high degree, solely in the external arachnoidal epi thelium; this pattern was found in one case with the variant visceral- restricted-type storage (confined to the cardiocytes). Thus, the exter nal arachnoidal epithelium may be particularly susceptible to Fabry li pid storage, probably caused by a distinctly larger sustained lysosoma l lipid load as compared to other cell types.