The Central Nervous System in Fabry's Disease
- 1 April 1973
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 28 (4) , 231-234
- https://doi.org/10.1001/archneur.1973.00490220039004
Abstract
An ultrastructural study of the central nervous system (CNS) in a case of Fabry's disease revealed intraneuronal inclusions of four types in the amygdala. These included zebra bodies and granulomembranous bodies similar to those seen in Hurler's syndrome, an inclusion made of concretions of delicate membranes and amorphous material, and lipofuscin bodies. Perithelial cells throughout the nervous system contained zebra bodies and granulomem-branous bodies, as well as inclusions resembling zebra bodies on end. Endothelial cells contained myelin ovoids. The inclusions seen ultrastructurally in the CNS differed from those seen in other regions of the body.Keywords
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