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To the Editor: In 1995, we reported proteinase K resistant prion protein (PrPres) in the brains of members of two families carrying a neurodegenerative disease originally classified as progressive subcortical gliosis (PSG).1,2 Of the four affected members of the first family examined, two (IV-9 and V-1) showed PrPres on immunoblot and cerebral areas that were immunoreactive to an antibody against PrP but that were Congo red negative and interpreted as"diffuse" plaques. One of the two patients (V-5) showed PrPres on immunoblot, although immunostaining was negative, and the other (V-3) was negative on both immunoblot and immunostain. Of the two affected members of the second family, the only one (II-7) who could be tested for PrPres by immunoblot had positive results, and immunocytochemistry was negative in both (II-6). Genetic analysis excluded mutations in the prion protein gene (PRNP) but showed a 15.8 odds ratio …
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