Although the etiology of ALS is largely unknown, the finding that familial ALS (fALS) is clinically indistinguishable from sporadic ALS and shares a common pathology of TAR DNA-binding protein 43 (TDP-43) proteinopathy supports the study of monogenetic causes to better understand the unifying pathogenic mechanisms to develop effective treatments (2). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.