PRNP and Creutzfeldt Jacob disease: In symptomatic IPD, PRNP mutations typically causing a rapid CJD-like disease phenotype (E200K, 4OPRI, E196K, V210I) were associated with very high concentrations of both tau and NfL in plasma, similar to those seen in sCJD, while the D178N mutation (typically causing either a CJD-like or fatal familial insomnia phenotype) gave slightly lower concentrations [28].