Editors' Note: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction. (6th September 2022)

• Record Type: Journal Article
• Authors: Siegler, James E.; Galetta, Steven
• Editors: Galetta, Steven; Ganesh, Aravind; Lewis, Ariane; Siegler, James E.
• Is Part Of: Neurology. Volume 99:Number 10(2022)
• Page Start: 447
• Page End: 447
• Publication Date: 2022-09-06
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/WNL.0000000000201153
• Languages: English
• ISSNs: 0028-3878

Abstract

Abstract : In their Teaching NeuroImage, Dr. Zhao et al. illustrate the case of a 15-year-old adolescent boy with acute flaccid upper extremity paralysis and a longitudinally extensive T2 spinal cord lesion. The vague maternal family history of optic neuropathy prompted the clinicians to test for m.14495T>C sequence variant, which confirmed the diagnosis of Leber hereditary optic neuropathy. Treatment with idebenone, a synthetic coenzyme Q analog that has approval by the European Medicines Agency (but is not approved by the Food and Drug Administration), was followed by resolution of symptoms within 3 months. In response, Dr. Irani and colleagues argue the radiographic diagnosis would be more consistent with an inflammatory myelopathy rather than a mimic of spinal cord infarction—as the authors postulate. Furthermore, they question whether immunotherapies were provided, which may have confounded the clinical improvement following idebenone. Dr. Zhao et al. confirm the patient's serum aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies were undetectable. Furthermore, the authors supplement the clinical history in their response to Dr. Irani with information that the 15-year-old patient had preceding bilateral optic neuropathy, which failed to respond to a pulse of methylprednisolone. Therefore, immunotherapies were not provided for this relapse. The patient's clinical improvement may only be attributable to the natural history of the condition or potentially the antioxidant effect of idebenone.