EARS2 and Leukoencephalopathy: For instance, mutations in DARS2 (MIM# 610956) (mitochondrial aspartyl-tRNA synthetase) have been identified as a recurrent cause of leukoencephalopathy with brainstem, spinal cord involvement, and lactate elevation, whereas EARS2 mutations (MIM# 612799) (mitochondrial glutamyl-tRNA synthetase) are associated with a leukoencephalopathy with thalamus and brainstem involvement and high lactate.