These findings raise the hypothesis that primary germline TARDBP mutations may be present in neurodegenerative diseases other than ALS and FTLD-U. Although no TARDBP mutations have been identified so far in those populations (Kabashi, et al., 2009,Rutherford, et al., 2008,Van Deerlin, et al., 2008), it is possible that the cohorts studied were underpowered.