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Developmental and Epileptic Encephalopathies (DEEs) are rare neurological disorders characterized by the onset of drug-resistant multiple epileptic seizures within the first years of life, and severe developmental delays and regressions [1]. Although very rare, DEE can be caused by mutations in genes involved in metabolic pathways crucial to various cellular functions in multiple organs including the brain. In recent years, defects in the mitochondrial malate aspartate shuttle (MAS) have been reported to be presenting the clinical phenotype of infantile epileptic encephalopathy [2–3].