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Adult PolysaccharidosisClinicopathological, Ultrastructural, and Biochemical Features
Nancy S. Peress, MD;
Salvatore DiMauro, MD;
Vivian A. Roxburgh, AS
Arch Neurol. 1979;36(13):840-845.
Abstract
• An abnormal polysaccharide in the form of cytoplasmic spheroids was found in the nervous system and systemic organs of a man with a progressive neurological disorder characterized by onset at 47 years of age, severe weakness, sensory loss, and dementia. Results of biochemical analysis showed a marked increase in brain and heart polysaccharide that was resistant to digestion by a mixture of glucosidases and that exhibited an iodine-complex spectrum higher than that of normal glycogen. Results of histochemical studies were consistent with the results of biochemical analysis and further defined the branching characteristics of the stored polysaccharide. Electron microscopy showed the cytoplasmic location of the spheroids, which were granular and filamentous.
Author Affiliations
From the Department of Pathology, State University of New York at Stony Brook and Veterans Administration Medical Center, Northport, NY (Dr Peress and Ms Roxburgh), and the Department of Neurology, College of Physicians and Surgeons of Columbia University, New York (Dr DiMauro).
Footnotes
Accepted for publication Oct 29, 1978.
Reprint requests to Department of Neuropathology, Room CO-45, Bldg 200, VA Medical Center, Northport, NY 11768 (Dr Peress).
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