Fig. 5From: Clinical and neuropathological phenotype associated with the novel V189I mutation in the prion protein geneNeuropathology of Case 1. The neuropathological analysis showed the presence of severe neuronal loss and spongiform changes in the cerebral cortex (a: frontal cortex, Haematoxylin-Eosin), associated with astrogliosis (b: frontal cortex, GFAP immunostaining). The pattern of PrPSc deposition was defined by diffuse, finely granular synaptic-like immunoreactivity (c: 3F4 immunostaining, frontal cortex). In the cerebellum, loss of Purkinje and very mild spongiosis in the molecular layer (d: Haematoxylin-Eosin), astrogliosis (e: GFAP immunostaining) and PrP build up were present: finely granular PrP deposits in the molecular layer and coarser spots in the granular layer (f: 3F4 immunostaining). The PrP deposits were not fluorescent after thioflavin S (not shown). Scale bars: in (a) = 100 μm (a, b, d and f are the same magnification); in (c) = 50 μm (c and e are the same magnification).Back to article page