Myoclonus-dystonia syndrome is a childhood-onset movement disorder related to mutations in the SGCE gene. 1 The gene encodes εsarcoglycan, a transmembrane protein with a brain-specific isoform. This genetic defect has autosomal dominant transmission with reduced penetrance because of maternal imprinting. 2 The myoclonus phenotype of SGCE-myoclonusdystonia has been extensively assessed in previous studies, including our own paediatric cohort. [3][4][5] In these studies, myoclonus has been reported to be more severe than dystonia. However, the dystonic phenotype has been evaluated mostly with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), a scale designed for primary generalized dystonia. Because dystonia in patients with SGCEmyoclonus-dystonia is predominantly task specific and shows a segmental distribution, there could have been a