Fig. 3
From: Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease
Neuropathological characterization. A1 Frontal cortex shows prominent neuronal loss and gliosis with loss of cortical structure and mild superficial spongiosis (H&E stain). A2 Anti-PrP immunohistochemistry (12F10 antibody) shows a very high density of multicentric PrP plaques covering the whole cortical thickness. A3 These large multicentric plaques are well identified in the PAS stain and the clusters of large amyloid plaques are observed by immunohistochemistry with the 12F10 anti-PrP antibody (A4). A5 Immunohistochemistry for hyperphosphorylated tau shows marked dystrophic neurites surrounding the plaques in the hippocampus (AT8 immunohistochemistry). A6 Abundant multicentric plaques are detected in the molecular layer of the cerebellum (anti-PrP immunostaining, 12F10 antibody)