Autosomal dominant mutations in CHCHD10 and its paralog CHCHD2 lead to a wide spectrum of neurodegenerative and neuromuscular diseases, including amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD, CHCHD10S59L), ALS (CHCHD10R15L), spinal muscular atrophy Jokela-type (SMA-J, CHCHD10G66V), autosomal dominant isolated mitochondrial myopathy and cardiomyopathy (IMMD, CHCHD10G58R) and Parkinson’s disease (CHCHD2T61I) [29–39]. This evidence concerns the gene CHCHD10 and frontotemporal dementia.