The phenotype of our patient with PSEN1 p.G209E was characterized with frontal symptoms such as personality change, temporal symptoms such as nonfluent languages, parietal symptoms such as agnosia and the presentation of extrapyramidal syndrome, which was more serious than the reported phenotype caused by G209R and G209V mutations inPSEN1 [27]. Here, PSEN1 is linked to agnosia.