The most common histopathological finding was frontotemporal lobar degeneration (FTLD) with TAR DNA binding protein 43 (TDP-43) inclusions (n = 8) with hippocampal sclerosis (n = 5/8), FTLD-tau (n = 4), mixed high likelihood Alzheimer’s disease and Lewy body disease (n = 4). The gene discussed is TARDBP; the disease is early-onset autosomal dominant Alzheimer disease.