Some of the most typical presentations are leukoencephalopathy with involvement of the brainstem and spinal cord and high lactate due to mt-aspartyl-tRNA synthetase (DARS2) mutations 150, leukoencephalopathy with thalamus and brainstem involvement and high lactate, caused by mt-glutamyl- tRNA synthetase (EARS2 151). The gene discussed is EARS2; the disease is Leukoencephalopathy.