TARDBP and amyotrophic lateral sclerosis: Supporting this notion, in the 63-year-old subject reported here with a small expansion of the HTT repeat, we also found typical signs of full-blown ALS pathology with atrophy of first and second motor neuron, accompanied by TDP-43-positive inclusions; but with only mild levels of polyQ- aggregates without striatal atrophy, in sum indicative of concomitant HD, which was, in contrast to the ALS disease, yet a still very early stage.