Recent evidence suggests that TDP-43 proteinopathy has been identified not only in CTE, but also in most cases of amyotrophic lateral sclerosis (ALS) [17], in a subset of the frontotemporal lobar degeneration (FTLD) with tau-negative ubiquitin-positive TDP-43-positive inclusions [18,19], and in specific disorders such as Alzheimer’s disease [20], Lewy body disease [21], hippocampal sclerosis [22], and corticobasal degeneration [20], suggesting its important role in the pathogenesis of neurodegeneration [23]. This evidence concerns the gene MAPT and amyotrophic lateral sclerosis.