Amyloid plaques in transmissible spongiform encephalopathies (prion diseases).
Amyloid plaques are encountered in all cases of kuru and Gerstmann-Straussler-Scheinker disease (GSS) and in some 10-15% of sporadic Creutzfeldt-Jakob disease (sCJD) cases. In variant Creutzfeldt-Jakob (vCJD) the particular type of plaque known as "florid" or "daisy" plaque exists in 100% of cases. By electron microscopy several types of amyloid plaque were delineated, corresponding to those seen by PrP immunohistochemistry. Unicentric "kuru" plaques consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a dense interwoven centre. A proportion of kuru plaques formed very dense stars, reminiscent of sea urchins in shape. Others presented a looser pattern. Amyloid stars were enveloped by astrocytic processes. High-power electron micrographs revealed that amyloid bundles were concealed within grooves of obscure cellular origin. Interestingly, basement membranes lined with electron-dense material were observed at the periphery of many amyloid plaques of GSS. Dystrophic neurites were seen only rarely. Microglial cells formed a significant part of the amyloid plaques. Occasionally clusters composed of several kuru plaques were found. These were intermediate forms to multi-centric plaques, which consisted of several merging stellate cores. Smaller amyloid deposits surrounded larger cores. In contrast to the kuru plaques, associated with a limited number of dystrophic neurites (DN), numerous such structures were seen at the periphery. The DN were filled with abnormal organelles such as electron-dense bodies, multi-vesicular bodies and multilamellar bodies and thus were indistinguishable from those seen in scrapie and CJD or Alzheimer's disease, except that they did not contain paired helical filaments (PHF). Instead, piled neurofilaments were often detected in the centres of DN. Similar DN were observed but these were not associated with any plaques. The last and, by the same token, the rarest type of plaque was the purely neuritic plaque. These consisted of large areas filled with DN of different sizes and shapes (sometimes bizarre) but not amyloid bundles. Analogously to the kuru and multi-centric plaques, astrocytic processes were observed at the periphery. By means of light microscopy and semi-thin (1 microm) sections discrete PrP-immunopositive plaques were observed (data not shown), in the subependymal region but not in the deep brain neuroparenchyma, in both 263K and 22C-H scrapie-infected hamster brains. These plaques were not discernible by routine haematoxylin and eosin staining. Ultrastructurally, plaques were recognised as areas of low electron density containing haphazardly-oriented fibrils which, when immunogold techniques were applied, were heavily decorated with PrP-conjugated gold particles.
Liberski
Paweł P
PP
Department of Molecular Pathology and Neuropathology, Medical University of Lódź, Poland. ppliber@csk.am.lodz.pl
D000818:Animals; D001921:Brain / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D046529:Microscopy, Electron, Transmission; D009474:Neurons / Q000648:ultrastructure*; D058225:Plaque, Amyloid / Q000648:ultrastructure*; D017096:Prion Diseases / Q000473:pathology*