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Adreno-leukodystrophy (Sex-Linked Schilder Disease)Ultrastructural Demonstration of Specific Cytoplasmic Inclusions in the Central Nervous System
Herbert H. Schaumburg, MD;
James M. Powers, MD;
Kinuko Suzuki, MD;
Cedric S. Raine, PhD
Arch Neurol. 1974;31(3):210-213.
Abstract
An ultrastructural study of the central nervous system of four male patients with adreno-leukodystrophy has been performed. Membrane-like cytoplasmic inclusions similar to those described in adrenal cortex, testis, and peripheral nerve were present in macrophages. These changes in widely scattered organs are probably the structural representation of a generalized metabolic disorder and are consistent with our previous hypothesis of a membrane-stability defect in these organs.
Author Affiliations
From the Saul R. Korey Department of Neurology (Dr. Schaumburg) and the Department of Pathology (Neuropathology) (Drs. Schaumburg, Suzuki, and Raine), Albert Einstein College of Medicine, Bronx, NY, and the Department of Pathology, Medical University of South Carolina-Veterans Administration Hospital, Charleston, SC (Dr. Powers).
Footnotes
Accepted for publication April 4, 1974.
Reprint requests to Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, Bronx, NY 10461 (Dr. Schaumburg).
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