The frequent co-occurrence (up to 75% of cases) of these proteinopathies may explain the excess of limbic atrophy compared to entorhinal atrophy at the early stages of Alzheimer’s disease.34 Indeed, pathological-MRI correlation studies showed that the hippocampus and the amygdala were the most frequently affected regions in TDP-43 positive brains and that the magnitude of atrophy mediated by TDP-43 inclusions was similar to tau-related atrophy.35 Here, TARDBP is linked to early-onset autosomal dominant Alzheimer disease.