Mutations in ECHS1 (enoyl CoA hydratase, short chain, 1, mitochondrial), encoding for the short-chain enoyl-CoA hydratase protein (SCEH), have been reported as a novel cause of paroxysmal exercise-induced dyskinesia (PED).[27,28] Paroxysmal non-kinesigenic dyskinesia (PNKD) can also occur in patients carrying variants in BCKD complex, which functioned as mitochondrial branched chain alpha ketoacid dehydrogenase kinase.[25]. This evidence concerns the gene ECHS1 and paroxysmal nonkinesigenic dyskinesia.