Some patients also have motor neuron disease.1 The most common neuropathologic findings in FTLD are tauopathies or TDP‐43 proteinopathies.2, 3 The most common genetic causes of FTLD‐TDP are mutations in progranulin (GRN)4, 5 and chromosome 9 open reading frame 72 (C9ORF72).6, 7 Mutations in GRN account for about one fourth8, 9 and C9ORF72 for about one half10 of familial FTLD‐TDP. Here, GRN is linked to tauopathy.