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Ultrastructure of Brain and Nerve Biopsy Tissue in Wilson Disease
Archinto P. Anzil, MD;
Heike Herrlinger, MD;
Karl Blinzinger, MD;
Albrecht Heldrich, MD
Arch Neurol. 1974;31(2):94-100.
Abstract
A 14-year-old girl with psychomotor deterioration developed Wilson disease. Mild atrophy of the small hand muscles was an unusual feature. A cerebral cortex biopsy specimen disclosed exuberant spheroid production, chromatolytic changes, myelin and axonal degeneration, alterations of capillary basal lamina, Hirano bodies, Alzheimer type protoplasmic astrocytosis, and many neuropil-based nonneuronal elements ("M" cells) of heterogeneous derivation distinct from astrocytes and normal oligodendrocytes. On reviewing neuroglia cell changes in light of present findings, the following conclusions become obvious: Alzheimer type II astrocytosis is true cellular hyperplasia of a class of reactive protoplasmic astrocytes with variable cytologic features. Alzheimer type I astrocytes most probably do not exist; the designation should be dropped. M cells play an important role in the cytopathology of Wilson disease; some forms may generate the so-called Alzheimer type I astrocyte.
Author Affiliations
Munich
From the Electron Microscopy Unit, Department of Neuropathology, Max Planck Psychiatric Institute (Drs. Anzil, Herrlinger, and Blinzinger), and the Second Pediatric Department, Munich-Schwabing Municipal Hospital (Dr. Heldrich), Munich.
Footnotes
Accepted for publication Oct 9, 1973.
Read before the 18th joint meeting of the Vereinigung Deutscher Neuropathologen und Neuroanatomen and the Scandinavian Neuropathological Society, Hamburg, Federal Republic of Germany, Sept 24, 1973.
Reprint requests to Max-Planck-Institut für Psychiatrie, Kraepelinstrasse 2, 8 München 40, Bundesrepublik Deutschland (Dr. Anzil).
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