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 leptomeningeal storage pattern, affecting all main leptomeningeal cell types (external arachnoideal epithelium, fibroblasts, vessel wall elements), was a consiste...

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Bibliographic Details
Published in:Acta neuropathologica 1994, Vol.88 (6), p.579-582
Main Authors: ELLEDER, M, CHRISTOMANOU, H, KUSTERMANN-KUHN, B, HARZER, K
Format: Article
Language:English
Subjects:
Aged
Arachnoid - metabolism
Arachnoid - pathology
Biological and medical sciences
Epithelium - metabolism
Epithelium - pathology
Errors of metabolism
Fabry Disease - pathology
Humans
Lipid Metabolism
Lipids (lysosomal enzyme disorders, storage diseases)
Male
Medical sciences
Metabolic diseases
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Summary: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.
ISSN:0001-6322
1432-0533
DOI:10.1007/BF00296496