Spinal cord abnormalities are rarely described in these neurogenetic disorders, with the exception of LBSL, which is a rare autosomal recessive mitochondrial disease that is associated with mutations in the DARS2 gene and characterized by a slowly progressive pyramidal and cerebellar dysfunction, presenting a distinct and uniform MRI pattern with signal abnormalities in the cerebral white matter and the selective involvement of specific brainstem and spinal-cord tracts (dorsal columns and lateral corticospinal tracts) [10]. This evidence concerns the gene DARS2 and inborn mitochondrial metabolism disorder.