TDP-43 pathology is frequently observed in the brains (18–60 %) of patients with DLB [2, 19, 37], however, tau, α-synuclein and Aβ amyloid deposits often coexist in these brains making it difficult to assess which of these primary insults may trigger TDP-43 inclusion formation. Here, TARDBP is linked to Lewy body dementia.