Laminar distribution of the pathological changes in sporadic and variant creutzfeldt-jakob disease

Abstract

The laminar distributions of the pathological changes in the cerebral cortex were compared in the prion diseases sporadic Creutzfeldt-Jakob disease (sCJD) and variant CJD (vCJD). First, in some cortical regions, the vacuolation ("spongiform change") was more generally distributed across the cortex in sCJD. Second, there was greater neuronal loss in the upper cortex in vCJD and in the lower cortex in sCJD. Third, the "diffuse" and "florid" prion protein (PrP(sc)) deposits were more frequently distributed in the upper cortex in vCJD and the "synaptic" deposits in the lower cortex in sCJD. Fourth, there was a significant gliosis mainly affecting the lower cortex of both disorders. The data suggest that the pattern of cortical degeneration is different in sCJD and vCJD which may reflect differences in aetiology and the subsequent spread of prion pathology within the brain.

Figure 1

Vacuolation in the upper cortical laminae of the frontal cortex in a case of sporadic Creutzfeldt-Jakob disease (sCJD). (cresyl violet, magnification bar = 200 μm).

Figure 2

Vacuolation and gliosis in the occipital cortex in a case of sporadic Creutzfeldt-Jakob disease (sCJD), (H/E, magnification bar = 50 μm).

Figure 3

Prion protein (PrP^sc) deposits in the frontal cortex of a case of variant Creutzfeldt-Jakob disease (vCJD), (PrP^sc immunohistochemistry, magnification bar = 50 μm).

Figure 4

Distribution of the prion protein (PrP^sc) deposits with distance across the cortical laminae in the cerebral cortex Creutzfeldt-Jakob disease (CJD). Diffuse deposits in variant CJD (vCJD), fit to polynomial (linear r = 0.82, P < .001); PrP^sc deposits in sporadic (CJD) (sCJD), fit to polynomial (second-order r = 0.81, P < .001).