TDP-43 immunoreactive histopathology has also been reported in 20–30% of patients with Alzheimer's disease (AD), 70% of patients with hippocampal sclerosis (HpScl), 33% of patients with Pick's disease and in a subset of patients with Lewy-body related diseases [8]–[12]. Here, TARDBP is linked to early-onset autosomal dominant Alzheimer disease.