The neuropathology is characterised by abundant neurofibrillary tangle (NFT) pathology, neuronal loss, amyloid plaques that resemble those observed in AD patients [2,5,6] together with alpha-synuclein pathology in the substantia nigra and cerebellum [7] and reactive glial cells and pathological TDP-43 positive inclusions in hippocampal neurons and motor neurons [8]. The gene discussed is TARDBP; the disease is Alzheimer disease.