Dementia observed in the patients with GHS exhibits characteristics similar to other types of age‐dependent dementing illnesses: (i) neuropathological analysis on the postmortem brain tissue of a GHS patient with dementia‐harboring TRIAD3 mutations (Margolin et al., 2013) revealed the presence of neuronal ubiquitin‐positive inclusions observed in patients with frontotemporal dementia (Rosso et al., 2001; Weder et al., 2007) and (ii) mutations in TRIAD3 were recently identified in patients suffering from Huntington‐like disease (HDL) with various symptoms including progressive dementia. The gene discussed is RNF216; the disease is frontotemporal dementia.