Pathology

Grossly, the brain is atrophic. The brunt of pathology is in the putamen and caudate, which are brownish, soft, and often cavitated. The histology is characterized by neuronal losses and a marked astrocytosis consisting chiefly of Alzheimer type 2 astrocytes. Macrophages are dispersed around the cavities; some contain lipids, and some contain pigments that stain positively for copper. A distinct finding is the presence of Opalski cells. These cells of macrophage/microglial origin are large, round, and have a small nucleus and finely granular eosino-philic cytoplasm. Similar, but less severe changes occur in the globus pallidus, thalamus, red nucleus, substantia nigra, and cerebellum. The cerebral cortex and white matter may show focal vacuolation and sponginess.

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