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September 1974

Adreno-leukodystrophy (Sex-Linked Schilder Disease): Ultrastructural Demonstration of Specific Cytoplasmic Inclusions in the Central Nervous System

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).

Arch Neurol. 1974;31(3):210-213. doi:10.1001/archneur.1974.00490390092013

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.