Mutations in the glutamyl-tRNA synthetase (EARS2, OMIM*612799) cause early onset severe neurological disease (leukoencephalopathy involving the thalamus and brainstem with high lactate, LTBL) and 8 out of 12 patients showed clinical improvement and stabilization after 1 year of age (10). This evidence concerns the gene EARS2 and Leukoencephalopathy.