Sequencing analyses of the 5 coding and 2 non-coding exons of TARDBP in our initial cohort of 176 clinical patients and 120 patients with pathologically confirmed TDP-43 pathology revealed 3 heterozygous missense mutations in 3 of the 116 analyzed ALS patients (2.6%), while no mutations were detected in 180 patients affected with FTLD-U, FTLD-MND, AD, HpScl and Lewy-body disease (Table 1, Figure 1). This evidence concerns the gene TARDBP and Alzheimer disease.