Leukoencephalopathy with brainstem and spinal cord involvement with elevated lactate (LBSL) is an unusual and rare leukoencephalopathy caused by autosomal recessive mutations in the DARS2 gene.58 Usually patients develop symptoms in childhood, but the condition tends to be slowly progressive and evolves over time, so the diagnosis is usually made when the patient is an adult. This evidence concerns the gene DARS2 and Leukoencephalopathy.