PSEN1 and visual agnosia: So far, over 300 PSEN1 mutations have been identified.1 Despite most carriers usually present with early amnestic symptoms, atypical cognitive, and non-cognitive symptoms have been described in patients with PSEN1, including frontotemporal dementia (FTD), early aphasia, visual agnosia, myoclonus and seizures, extrapyramidal features, and spasticity (Riudavets et al., 2013; Tang et al., 2016).