PMID: 11928893Apr 4, 2002Paper

A special report I. Prion protein (PrP)--amyloid plaques in the transmissible spongiform encephalopathies, or prion diseases revisited

Folia Neuropathologica
P P LiberskiP Brown

Abstract

We present a retrospective analysis of PrP-amyloid plaques encountered in CJD and GSS. In human TSEs (kuru, CJD and GSS) several PrP-immunopositive plaques and plaque-like deposits were detected. In kuru, plaques were typical "kuru" plaques--stellate structures deposited mostly in the granular- and Purkinje-cell layer of the cerebellum. Many smaller or larger clusters were visible but, in contrast to GSS, they never merged together to form multicentric plaques. In all cases of GSS, plaques were localised in the granular- and Purkinje-cell layer and the molecular cell layer. There were many different forms of plaques: from kuru plaques (unicentric stellate plaques) to clusters of unicentric plaques, which by merging eventually formed "multicentric plaques". The latter are the hallmark of this disease. By electron microscopy, several types of amyloid plaques, which corresponded to those seen by PrP immunohistochemistry, were observed. The first type, unicentric "kuru" plaque, consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a densely interwoven centre. Amyloid stars were surrounded by astrocytic processes and invaded by microglial cells but dystrophic neurites were only rarely seen. In contras...Continue Reading

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