TDP-43 pathology was identified in the anterior cingulate cortex of 87% ALS (n = 13) and all other cases, and in the motor cortex of 93% ALS (n = 14), 86% FTLD (n = 24) and 100% FTLD-ALS cases (n = 18). Here, TARDBP is linked to amyotrophic lateral sclerosis.