TBK-1 mutations were first identified in ALS in 2015 (Cirulli et al., 2015), and since then more than 40 mutations have been identified, representing 1.3%–3.4% in patients with ALS (Cirulli et al., 2015), 0.4%–1.1% in patients with FTD and 3.6%–4.5% of ALS-FTD cases (Gijselinck et al., 2015). Here, TBK1 is linked to frontotemporal dementia.