Probable Adult Polyglucosan Body Disease

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Presentation transcript:

Probable Adult Polyglucosan Body Disease Caroline M. Klein, MD, PhD, E. Peter Bosch, MD, Peter J. Dyck, MD Mayo Clinic Proceedings Volume 75, Issue 12, Pages 1327-1331 (December 2000) DOI: 10.4065/75.12.1327 Copyright © 2000 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 1 T2-weighted magnetic resonance images. A, Axial image demonstrates hyperintense confluent white matter lesions in periventricular frontal and occipital regions. B, Sagittal image shows prominent cervical spinal cord atrophy. Mayo Clinic Proceedings 2000 75, 1327-1331DOI: (10.4065/75.12.1327) Copyright © 2000 Mayo Foundation for Medical Education and Research Terms and Conditions

Fig. 2 Suralnervebiopsyspecimens revealedprominent large polyglucosan bodies. A, Longitudinal paraffin-embedded section stained withLuxolfast blue/periodic acid-Schiff demonstrates an enlarged polyglucosan body separating the myelin sheath of a myelinated fiber (arrow). B, Semithin epoxy-embedded cross section of a largepolyglucosan bodystainedwithmethylene blue surrounded by thinned myelin(arrow). C, Transmission electron micrograph of a typical polyglucosan bodydemonstrating a separation between the edge of the polyglucosan body and normalappearing axoplasm (arrow). Note the overlapping fibrilsembedded withina densebackground matrixmaterial. Mayo Clinic Proceedings 2000 75, 1327-1331DOI: (10.4065/75.12.1327) Copyright © 2000 Mayo Foundation for Medical Education and Research Terms and Conditions