ISSN:
1432-0533
Keywords:
Fabry disease
;
Leptomeningeal storage
;
External arachnoideal epithelium
;
Restricted type of visceral storage
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract 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 particurlarly susceptible to Fabry lipid storage, probably caused by a distinctly larger sustained lysosomal lipid load as compared to other cell types.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00296496
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