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

1st Institute of Pathology School of Medicine Charles University Prague Czech Republic

See more in PubMed

Int J Dev Neurosci. 1990;8(2):175-85 PubMed

Acta Neuropathol. 1982;56(2):93-8 PubMed

Am J Pathol. 1978 Jul;92(1):53-68 PubMed

Cell Tissue Res. 1993 Aug;273(2):209-17 PubMed

Hum Genet. 1993 Sep;92(2):143-52 PubMed

Arch Neurol. 1971 Oct;25(4):351-9 PubMed

N Engl J Med. 1991 Feb 7;324(6):395-9 PubMed

Z Zellforsch Mikrosk Anat. 1967;79(2):272-95 PubMed

Am J Pathol. 1978 Jul;92(1):69-84 PubMed

Virchows Arch A Pathol Anat Histopathol. 1990;417(5):449-55 PubMed

J Lipid Res. 1990 Feb;31(2):335-40 PubMed

Ann Neurol. 1988 May;23(5):505-9 PubMed