Therapeutic possibilities in CJD: patents 1996-1999

Currently, there is no effective treatment for Creutzfeldt-Jakob disease (CJD). However, an increasing understanding of the underlying disease mechanisms has suggested a number of therapeutic possibilities, many of which are registered as patents. The fundamental disease mechanisms in CJD are the conversion of PrPC to PrPSc, and the aggregation and deposition together under three main headings: reducing the production of PrPC; prevention of the post-translational alteration of PrPSc; prevention of neurotoxicity of PrPSc and amyloid deposits. The scientific background to these proposals is outlined and their claims are critically reviewed. In general, the treatment suggestions are based on theory, in vitro experiment and animal model data, with little, if any, definitive evidence of efficacy or safety in human disease.