TARDBP and amyotrophic lateral sclerosis: The FTLD-TDP type A is defined as the presence of many small compact or crescent neuronal cytoplasmic TDP-43 inclusions and short dystrophic neurites primarily in the cortical layer 2, which is associated with non-fluent primary progressive aphasia (PPA) and the behavioral variant FTLD (bvFTLD) (with or without ALS) [3,179].