TARDBP and amyotrophic lateral sclerosis: Although most cases of clinical PLS have cortical ubiquitin and TAR DNA-binding protein 43 (TDP-43) pathology [3, 4] and the PLS phenotype may appear as part of the phenotypic spectrum in familiar ALS pedigrees [5], PLS is no more considered part of the ALS spectrum by the Gold Coast Criteria [6].