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  Vol. 28 No. 4, April 1973 TABLE OF CONTENTS
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The Central Nervous System in Fabry's Disease

An Ultrastructural Study

Margaret L. Grunnet, MD; Paul R. Spilsbury, MD

Arch Neurol. 1973;28(4):231-234.


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.



Author Affiliations

Salt Lake City

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


Footnotes

Accepted for publication Oct 3, 1972.

Reprint requests to departments of neurology and pathology, University of Utah College of Medicine, 3E 512 Medical Center, Salt Lake City 84112 (Dr. Grunnet).



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THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

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Quantitative analysis of epidermal innervation in Fabry disease
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Neurology 1999;52:1249-1249.
ABSTRACT | FULL TEXT  





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