CBS is more heterogeneous with the following underlying pathological substrates observed: corticobasal degeneration (CBD) , progressive supranuclear palsy (PSP), frontotemporal lobar degeneration (FTLD)-Tau (Pick’s disease) and FTLD-Ubiquitin/TAR DNA binding protein (TDP-43), Alzheimer’s disease (AD) and, rarely, DLB [2–6]. The gene discussed is TARDBP; the disease is early-onset autosomal dominant Alzheimer disease.