Human prion diseases in The Netherlands : clinico-pathological, genetic and molecular aspects

Prion diseases, or transmissible spongiform encephalopathies (TSEs), are invariably fatal neurodegenerative disorders that can be sporadic, inherited or acquired by infection. In humans, TSEs comprise three major groups showing a wide phenotypic heterogeneity: Creutzfeldt-Jakob disease (CJD), Gerstmann-Str䵳sler-Scheinker (GSS) disease and fatal insomnia (FI). In The Netherlands, a national surveillance program was established in 1997 to monitor the incidence of both existing and emerging forms of prion diseases, in particular new variant CJD (vCJD), which is causally related to bovine spongifo... Mehr ...