Cognitive and neuropsychiatric features of ALS have largely been attributed to changes in the frontotemporal and insular cortex (Tsujimoto et al., 2011; Agosta et al., 2016; Westeneng et al., 2016; Consonni et al., 2018a,b; Christidi et al., 2018a), although increasing evidence suggest hippocampus and subcortical region degeneration also plays a role (Bede et al., 2013b; Machts et al., 2015; Westeneng et al., 2016), especially in C9+ALS patients (Bede et al., 2013a; Agosta et al., 2017; Floeter et al., 2018; Floeter and Gendron, 2018). The gene discussed is C9; the disease is amyotrophic lateral sclerosis.