Strammiello, R.

disease prion mutation patient amyloid protein cortex prion protein prpsc neuro fibrillary tangles brain plaque neuropathol deposit prion disease antibody prion protein gene amyloid angiopathy cerebellum codon netherland fragment creutzfeldt-jakob disease dementia phenotype creutzfeldt-jakob analysis neuro residue fibrillary accumulation prion diseases y 226x mutation q 227x mutation ??? patient 2 tangle angiopathy disease duration frontotemporal dementia neurol change deposition fi ndings centre prp-caa copyright © 2011 blood vessels g 131v mutation nding american association parchi gss disease association tau accumulations novel amyloid depositions examination american codon mutations result neuropathologist month prion protein amyloidoses feature tissue gerstmann-straussler-scheinker ¨ disease neuro fibrillary lesions 8- kd fragment striatum +++ multicentric amyloid plaques blood patient 1 vessel amyloid deposits purkinje cells thalamu gerstmann-straussler-scheinker disease tau deposits