CHCHD10-related diseases include mtDNA instability disorder, the clinical spectrum of frontotemporal dementia–amyotrophic lateral sclerosis (FTD-ALS), late-onset spinal motor neuropathy (SMAJ), and Charcot–Marie–Tooth disease type 2 (CMT2) [184,215,216]. This evidence concerns the gene CHCHD10 and frontotemporal dementia.