The rare heterozygous variants in PSEN2 identified in this work, namely c.772G>A (p.Ala258Thr) and c.1073-2_1073-1del, may be genetic alterations that predispose to or cause dementia, with this clinical pattern mimicking FTD (behavioral–language) and showing congruent neuroimaging of frontal impairment, though more cases are needed to confirm this hypothesis. This evidence concerns the gene PSEN2 and frontotemporal dementia.