TDP-43 seeding was observed in 9 OM (56%) of patients with genetic MND associated with TDP-43 pathology, including 2/4 with TARDBP (50%), 2/3 with SQSTM1 (67%), 1/1 with OPTN (100%), 1/1 with GLE1 (100%), and 3/6 with C9orf72exp (50%). Here, SQSTM1 is linked to mild neurocognitive disorder.