Amyotrophic lateral sclerosis (ALS), half of the cases of frontotemporal lobar degeneration (FTLD) and the recently discovered limbic-predominant age-related TDP-43 encephalopathy (LATE) are associated with a common histopathology based on the presence of cytoplasmic inclusions of the nuclear TAR DNA-binding protein 43 (TDP-43) in neurons and motoneurons of the central nervous system, accompanied by a concomitant disappearance of the soluble protein from the nucleus of the same cells [1–5]. This evidence concerns the gene TARDBP and frontotemporal dementia.