Reader Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction

We thank Zhao et al.(1) for presenting an interesting case of a longitudinally extensive spinal cord lesion in a patient with a mitochondrial sequence variant. The authors suggest that the neuroimaging mimics spinal cord infarction (SCI). However, the initial MRI shows a swollen, expanded, and enhancing spinal cord lesion. This appearance is typical for neuromyelitis optic spectrum disorders or other inflammatory myelopathies, rather than SCI. While acute snake eye appearance suggests SCI, it is usually nonenhancing, and an early MRI could be normal.(2) In addition, evidence for the efficacy of idebenone for the treatment of optic neuropathy in Leber Hereditary Optic neuropathy (LHON) is modest at best,(3) making it difficult to attribute this patient's recovery to idebenone. Was the patient treated with steroids or another immunotherapy acutely? In our opinion, it is likely that this patient, who harbors a familial mitochondrial sequence variant, presented with inflammatory myelopathy. Association of LHON and multiple sclerosis or antiaquaporin-4 disease is known and likely responsible for the aggressive presentation here(4,5); however, causality is not proven. While we agree with the authors that consideration of underlying mitochondrial disorders in the right clinical scenario is important, ongoing neuroimmunology surveillance is prudent, and on relapse, the case may warrant immunotherapy.