The presence of abnormal TDP43 protein deposition is a pathological feature of motor neuron disease (MND) and frontotemporal lobar degeneration with TDP43-positive inclusions (FTLD-TDP) and a variety of FTLD-TDP subtypes are recognized based on the morphology and anatomical distribution of both neuronal inclusions and dystrophic neurites [2–4] (Figure 1). Here, TARDBP is linked to frontotemporal dementia.