Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene.


The use of prion protein (PrP) immunohistochemistry in neuropathology has allowed identification of prion diseases with otherwise atypical histological features. The brains from family members with familial prion diseases can show marked histological variation. A histological and immunohistochemical study was performed on 10 brains of patients with a… (More)


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