摘要

Objective To quantify cortical white matter pathology in variant Creutzfeldt-Jakob disease (vCJD) and to correlate white and grey matter pathologies Methods Pathological changes were studied in immunolabeled sections of the frontal, parietal, occipital, and temporal cortex of eleven cases of vCJD Results Vacuolation ("spongiform change"), deposition of the disease form of prion protein (PrP(sc)), and a glial cell reaction were observed in the white matter The density of the vacuoles was greatest in the white matter of the occipital cortex and ghat cell density in the inferior temporal gyrus (ITG) Florid-type PrP(sc) deposits were present in approximately 50% of white matter regions studied In the white matter of the frontal cortex (FC), vacuole density was negatively correlated with the densities of both glial cell nuclei and PrP(sc) deposits In addition, in the frontal and parietal cortices the densities of glial cells and PrP(sc) deposits were positively correlated In the FC and ITG, there was a negative correlation between the densities of the vacuoles in the white matter and the number of surviving neurons in laminae V/VI of the adjacent grey matter In the FC, vacuole density in the white matter was negatively correlated with the density of the diffuse PrP(sc) deposits in laminae II/III and V/VI of the adjacent grey matter In addition, the densities of PrP(sc) deposits in the white matter of the FC were positively correlated with the density of the diffuse PrP(sc) deposits in laminae II/III and V/VI and with the number of surviving neurons in laminae V/VI Conclusion The data suggest significant degeneration of cortical white matter in vCJD, the vacuolation being related to neuronal loss in the lower cortical laminae of adjacent grey matter, PrP(sc) deposits the result of leakage from damaged axons, and gliosis a reaction to these changes

  • 出版日期2010-12