MOG-Positive Optic Neuritis In A Child Presenting With Bilateral Disc Edema And Pre-Retinal Hemorrhage

Abstract

Abstract Title: MOG-Positive Optic Neuritis In A Child Presenting With Bilateral Disc Edema And Pre-Retinal Hemorrhage Rasika Sudharshan1, Mark S. Borchert2, Melinda Y. Chang2 1USC Keck School of Medicine, 2Children’s Hospital Los Angeles, Department of Ophthalmology Background Our case discusses an 8-year-old male with no relevant past medical history or trauma, who presented with two weeks of progressive bilateral vision loss that acutely worsened over 3 days. This case demonstrates typical features of optic neuritis in children, including bilaterality, severe vision loss, disc edema, and the atypical finding of extensive retinal and preretinal hemorrhages in the macula. Case Presentation On presentation, his visual acuity was light perception with dilated and poorly reactive pupils. Fundus exam showed bilateral optic disc edema (with pallor) and retinal venous tortuosity without cotton wool spots. Pupil exam showed a right RAPD. His left fundus exam was also significant for preretinal and intraretinal hemorrhages. Lumbar puncture showed an elevated opening pressure (36mmH2O) with normal glucose, abnormal protein at 77, and mildly elevated pleocytosis at 12. Brain MRI showed an incidental small posterior fossa arachnoid cyst but no white matter lesions, and orbit MRI showed bilateral optic nerve enhancement consistent with optic neuritis, right greater than left. Lab tests showed a normal CBC and a MOG-Ab positive titer level of 1:100. After six doses of IV methylprednisolone and completion of five days of plasmapheresis, visual acuity improved to 10/200 OD and 20/100 OS. Then, after three rounds of IVIG and prednisone taper, his visual acuity improved to 20/80 and 20/30. Forty-eight days after treatment, bilateral optic disc edema and retinal hemorrhages resolved. His final examination showed bilateral optic atrophy OU and visual acuity of 20/40 OD and 20/20 OS. Discussion Due to increased intracranial pressure (ICP) on lumbar puncture and intra- and preretinal hemorrhages, papilledema or leukemia were considered. Increased ICP has not been consistently reported in patients with MOG-Ab ON, and ICP was believed to be falsely elevated in this case due to excessive hypercapnia associated with sedation. Retinal hemorrhages are rare in optic neuritis, which typically occurs without disc edema in adults. In children, optic neuritis may be associated with disc edema and peripapillary hemorrhages in severe cases, especially in those with MOG-Ab positivity. This is the first case, to our knowledge, of preretinal hemorrhage in the macula associated with MOG-Ab positive optic neuritis.