Cytoplasmic neuronal and glial inclusions of TDP-43 are found in 98% of all cases of ALS (sporadic and familial) and in the majority of Tau-negative frontotemporal lobar degeneration (FTLD-TDP) cases, typically behavioral variant frontotemporal dementia (FTD) [5]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.