Plasma EV TDP-43 discriminated bvFTD cases from HC, PSP, ALS and ALS–FTD (AUC sEV: bvFTD versus HC 0.87, CI [0.803–0.926]; versus PSP 0.91, CI [0.851–0.959]; versus ALS 0.76, CI [0.687–0.832]; versus ALS–FTD 0.82, CI [0.745–0.881]) (Extended Data Fig. 6i and Supplementary Table 14 (sEV), and Supplementary Fig. 18h–j and Supplementary Table 14 (mEV)). Here, TARDBP is linked to behavioral variant of frontotemporal dementia.