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April 1973

The Central Nervous System in Fabry's Disease: An Ultrastructural Study

Author Affiliations

Salt Lake City
From the departments of neurology and pathology, University of Utah College of Medicine, Salt Lake City.

Arch Neurol. 1973;28(4):231-234. doi:10.1001/archneur.1973.00490220039004

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.

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