An autopsy report of three kindred in a Gerstmann-Sträussler-Scheinker disease P105L family with a special reference to prion protein, tau, and beta-amyloid.

The clinicopathological diversity of GSS105, which is possible even in the same family, was ascertained. Not only p-tau but also Aβ could be induced by PrP ("secondary degeneration"), facilitating the kaleidoscopic symptoms of GSS.