Expanding the electro-clinical phenotype of CARS2associated neuroregression

•Pathogenic variants in cysteinyl-tRNA synthetase-2 (CARS2) gene have recently been described to cause severe myoclonic epilepsy, neuroregression, progressive audio-visual impairment and movement disorder.•We present an 11-year-old boy with neuroregression, dysfluent speech, aggressive behavior, tremors and ESES pattern on EEG•Our case expands the existing electro-clinical phenotype of CARS2 associated neuroregression.