Nanoparticle Tracking Analysis (NTA) data are obtained from EVs isolated from 172 human plasma samples, from patients with sporadic FTLD (n=40), with FTLD due to C9orf72 expansion (n = 9 C9orf72 intermediate expansion carriers; n = 31 C9orf72 pathological expansion carriers), with FTLD due to pathogenic mutations in GRN (n = 46 heterozygous mutation carriers), with NCL due to pathogenic mutation in GRN (n= 3 GRN homozygous null mutation carriers) and subjects with normal cognitive function, as control group (n = 43). Moreover, a negative control was included in the analysis (ctrl_neg). Samples were analyzed with Nano-Sight NS300 Instrument (Malvern, Worchestershire, UK). For each sample, 5 video of 60 seconds duration were recorded and data were processed using NanoSight NTA Software 3.2 (Malvern, Worchestershire, UK). Correspondence_RawData table shows sample code, category as diagnostic group, age and the corresponding raw file name. Raw data of each sample analyzed consists of .csv file generated by Nano-Sight NS300 Instrument. File contains information about EVs concentration (particles/ml, particles/frame), size (mean, mode, SD) and size distribution (D10, D50 and D90). Representative video of each main diagnostic group are available in the NTA_Video folder.