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Leptomeningeal lipid storage patterns in Fabry disease

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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.

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Authors and Affiliations

  1. 1st Institute of Pathology, School of Medicine, Charles University, Studničkova 2, CR-12800, Prague 2, Czech Republic

    M. Elleder

  2. Oceanographic Biochemical Society, Lampsakou 2, Athens, Greece

    H. Christomanou

  3. Laboratory of Neurochemistry, Institute of Brain Research, University of Tübingen, Germany

    B. Kustermann-Kuhn & K. Harzer

Authors
  1. M. Elleder
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  2. H. Christomanou
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  3. B. Kustermann-Kuhn
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  4. K. Harzer
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Elleder, M., Christomanou, H., Kustermann-Kuhn, B. et al. Leptomeningeal lipid storage patterns in Fabry disease. Acta Neuropathol 88, 579–582 (1994). https://doi.org/10.1007/BF00296496

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  • DOI: https://doi.org/10.1007/BF00296496

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