Sassi et al. (2021) performed whole exome sequencing in 100 patients with a late-onset and heterogeneous FTD-like clinical phenotype and identified two sporadic cases with behavioral FTD carring heterozygous CSF1R p.E573K and p.R549H mutations. Sharma et al. (2019) reported an HDLS patient carrying a known pathogenic variant [2381T > C (p.Iso794Thr)] in the CSF1R gene but presenting clinical symptoms and brain imaging features similar to that of Dementia with Lewy Bodies (DLB). This evidence concerns the gene CSF1R and Lewy body dementia.