DYT-KMT2B appears to be one of the causes of dystonia with the highest risk of developing status dystonicus together with pantothenate-kinase associated neurodegeneration and GNAO1-related movement disorders compared to other monogenic childhood-onset dystonias such as DYT-TOR1A and THAP1-related dystonia which may be another helpful distinguishing disease feature (Opal et al., 2002; Ben-Haim et al., 2016; Koy et al., 2018; Nerrant et al., 2018; Oterdoom et al., 2018; Waak et al., 2018; Schirinzi et al., 2019). This evidence concerns the gene KMT2B and Dystonia.