TARDBP and behavioral variant of frontotemporal dementia: As in DESCRIBE subcohort 2, plasma EV TDP-43 levels were increased in patients with ALS (median sEV TDP-43: 45.60 pg ml−1, IQR [31.55–64.45]) compared with HC (median sEV TDP-43: 10.41 pg ml−1, IQR [8.50–14.65], P < 0.00001), in ALS–FTD (median sEV TDP-43: 52.40 pg ml−1, IQR [39.18–73.43], P < 0.00001 compared with HC) and in bvFTD (median sEV TDP-43: 24.15 pg ml−1, IQR [11.13–40.55], P < 0.00001 compared with HC) (Fig. 5h (sEV), and Supplementary Fig. 19a and Extended Data Table 3 (mEV)).