The main differences between ALS/FTD patients and classical FTD cases are presences of psychiatric signs and the distribution of TDP-43 inclusions: SALS primarily features TDP-43 pathology in the spinal cord, patients with FTD primarily affect the cortex, while FTD-ALS patients have TDP-43 pathology in both areas (Geser et al., 2009; Neumann et al., 2009). The gene discussed is TARDBP; the disease is frontotemporal dementia.