NR AOVW
AU Liberski,P.P.; Streichenberger,N.; Giraud,P.; Soutrenon,M.; Sikorska,B.; Kopp,N.
TI Pathology of prions revisited: a brain biopsy studies
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-13
PT Konferenz-Poster
AB We report here a detailed ultrastructural study of series of brain biopsies from cases of CJD, and from one case of FFI. The latter disease has not heretofore benefited from ultrastructural study. In particular, we searched for tubulovesicular structures, 35-nm particles regarded as the only disease-specific structures at the level of thin-section electron microscopy. Our material consisted of brain biopsies obtained by open surgery from 1 FFI case from a new French family, 1 case of vCJD, 9 cases of sporadic CJD, 2 cases of iatrogenic (human growth hormone) CJD and 2 cases of hereditary CJD (Gly200Lys and Val203Iso). The ultrastructural picture of the cerebral cortex of the FFI patient was virtually indistinguishable from that of Creutzfeldt-Jakob disease (CJD). Typical spongiform change was observed, consisting of intracellular membrane-bound vacuoles containing secondary chambers (vacuoles within vacuoles) and amorphous material. Neuronal degeneration was widespread: some processes contained degenerating mitochondria and lysosomal electron-dense bodies and these met the criteria for neuroaxonal dystrophy. Other processes contained branching cisterns; still others were filled with electron-dense masses and amorphous vesicles. Tubulovesicular structures (TVS) were found, albeit only after prolonged search. The overall ultrastructural appearance of variant CJD was not much different than that of FFI cerebral cortex but hypetrophic astrocytes were readily observed. The only exception was much higher number of cellular processes containing TVS. For sporadic CJD, we could detect TVS in the majority of cases. While typical spongiform change and robust astrocytic reaction were seen, additional features were observed. These include, widespread neuritic and synaptic degeneration and autophagic vacuoes. In conclusion, we showed that TVS are readily found in FFI, vCJD and sCJD and that widespread neuritic degeneration is a part of ultrastructural pathology in prions.
AD Pawel P. Liberski, Beata Sikorska, Dept. Mol. Pathol., Med. Univ. Lodz; Nathalie Streichenberger, Pierrie Giraud, Mireille Soutrenon, Nicolas Kopp, Hopital Neurologique et Neuro-chirurgical Pierre Wertheimer; Anatomie Pathologique et de Neuropathologique, Lyon, France
SP englisch
PO Deutschland