Although both cases showed a high burden of neuritic plaques, the neuronal component of the neuritic plaques was negative for phospho-tau and AD-type tau pathology was negligible, suggesting that rather of having a coincidental Alzheimer’s disease (that requires both neuritic plaques and phospho-tau pathology), it is possible that Aβ pathology in this family is part of the same process leading to TDP-43 pathology. The gene discussed is TARDBP; the disease is Alzheimer disease.