TDP-43 pathology was first discovered in 2006 as a primary pathological feature of frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP), amyotrophic lateral sclerosis (ALS), and later in hippocampal sclerosis with amnestic cognitive impairment not associated with FTLD/ALS (Amador-Ortiz et al. 2007). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.