The radiological appearances of patients with LS harboring the m.13094T>C mutation bear similarities to mitochondrial diseases caused by autosomal recessive tRNA synthetase mutations, such as leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL) caused by EARS2 mutations and leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) caused by DARS2 mutations (Diodato et al., 2014). Here, EARS2 is linked to mitochondrial disease.