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 (PrPsc) 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.

A Review of Neuroimaging in Rare Neurodegenerative Diseases

“…The iatrogenic CJD (iCJD) is transmitted by growth hormone acquired from pituitary glands, corneal transplant, or dura mater grafts. The most common subtype is sporadic CJD (sCJD) which develops due to sporadic mutation or post-translational modification of PrP gene [33] . Recently, another subgroup came in the spotlight, described in the UK, the variant CJD (vCJD) which is a result of consumption of meat from cattle with bovine spongiform encephalopathy and differs clinically from other subtypes [34].…”

Original article Spatial relationships between diffuse prion protein deposits and neuronal perikarya in variant Creutzfeldt-Jakob disease

“…The distribution of diffuse deposits in the cortex in vCJD varies with laminar depth [11]. Hence, to investigate the correlation between the size of a diffuse PrP sc deposit and frequency of embedded neuronal perikarya, guidelines were marked on the slide extending from the pia mater to the white matter.…”