Importantly, all bvFTD TDP-43 pathology cases were in the lower range, comparable with HC, and ALS PSP/GGT-type 4R tau pathology cases were characterized by decreased plasma EV 3R/4R tau levels (median sEV 0.42, IQR [0.35–0.60]) compared with HC (median sEV 0.99, IQR [0.91–1.03], P < 0.00001), with TDP-43 pathology (median sEV 0.95, IQR [0.92–0.97], P < 0.00001) and with non-TDP43/non-tau pathology groups (median sEV 0.96, IQR [0.90–1.03], P < 0.00001). Here, MAPT is linked to amyotrophic lateral sclerosis.