However, so far no consistent correlation of RNA foci and DPR protein pathology with the regional pattern of neurodegeneration and/or presence of TDP-43 pathology, the neuropathological hallmark feature of ALS and FTD including cases with C9orf72 mutations [36], has emerged despite extensive quantitative analysis [11, 12, 26, 27]. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.