While in ALS with TDP-43 inclusions (ALS-TDP), TDP-43 pathology is primarily observed within neurons and glia in the spinal cord, motor cortex and brainstem motor nuclei [34], the distribution and morphology of TDP-43 inclusions in FTD with TDP-43 inclusions (FTD-TDP), particularly in C9ORF72 mutation carriers, is more heterogenous [35, 36, 46]. This evidence concerns the gene TARDBP and frontotemporal dementia.