This clinical overlap [6] is reflected at the neuropathological level, as both syndromes share a common hallmark, that is, the accumulation of TAR DNA‐binding protein (TDP‐43) [7] type A and B [8], whose aggregates underlie around 50% of bvFTD [9] (with tau pathology representing the second most frequent neuropathological correlate [10]) and the majority of ALS [11] cases. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.