One of the key pathological hallmarks of MND (both sporadic and familial) is the presence of TDP-43 inclusions in motor neurons [9, 10], although not in SOD1-linked MND [10] In familial MND with mutations in the TARDBP gene (encoding TDP-43), the disease-causing mutations cluster in the TDP-43 C-terminal region [4]. Here, TARDBP is linked to mild neurocognitive disorder.