The spatial pattern of the vacuolation in patients with sporadic Creutzfeldt-Jakob disease

The spatial pattern of the vacuolation ('spongiform change') was studied in the upper and lower laminae of the cerebral cortex, the CA1/CA2 sectors of the hippocampus and the molecular layer of the cerebellum in 11 cases of s poradic Creutzfeldt-Jakob disease (CJD). Individual vacuoles were grouped i nto clusters, 50 to >1600 mu m in diameter and, in the majority of tissue s ections, the vacuole clusters were distributed with regular periodicity par allel to the tissue boundary. The size of the vacuole clusters was positive ly correlated with patient age in the lower laminae of the occipital cortex a nd the inferior temporal gyrus (ITG) a nd negatively correlated with age in the hippocampus. In addition, the size of the vacuole clusters was posi tively correlated with disease duration in the upper laminae of the ITG. Th e size and distribution of the vacuole clusters suggests that the vacuolati on in CJD reflects the degeneration of specific brain pathways and supports the hypothesis that prion pathology may spread th rough the brain along we ll defined anatomical pathways. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.