Importantly, Guamanian ALS/PDC is associated with tar DNA-binding protein 43 (TDP-43) proteinopathies similar to frontotemporal lobar degeneration with ubiquitin positive inclusions (FTLD-U) with/without motor neuron disease (MND) as well as ALS, and neocortical or hippocampal TDP-43 pathology which distinguishes controls from disease subjects in these entities better than tau pathology. The gene discussed is TARDBP; the disease is frontotemporal dementia.