Outcomes of Children With Optic Neuritis as Their First Demyelinating Event

Abstract

Introduction

Optic neuritis (ON) is an acquired demyelinating syndrome and the most common cause of acute optic nerve inflammation in children and adults. This study describes the clinical and diagnostic features, visual outcomes, and relapse risk of children presenting with their first ON episode.

Methods

We prospectively identified 50 children presenting with ON as their first demyelinating event. Patients underwent both serum myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibody screening (n = 42) or were diagnosed with AQP4-seropositive neuromyelitis optica spectrum disorder (NMOSD) without MOG testing (n = 8). We analyzed demographics, antibody status, magnetic resonance imaging (MRI) findings, treatments, relapses, and visual disability.

Results

Subjects were stratified by diagnosis into idiopathic ON (n = 6), MOG antibody disease (n = 20), multiple sclerosis (n = 11), and NMOSD (n = 13). Females comprised 66% of the cohort. The mean age at onset was 12 years, and Black patients represented 48% of the cohort. Decreased visual acuity was nearly universal (96%). The logMAR of the worst eye at onset was most severe in NMOSD (3.1) and mildest in idiopathic ON (1.3). Ninety percent received intravenous steroids as acute treatment. Visual recovery varied by diagnosis, with mean improvement of 1.5 logMAR. Half (n = 25) experienced relapses, most commonly ON (19 of 25) or longitudinally extensive transverse myelitis (9 of 25). NMOSD patients had the highest relapse rates and poorest visual outcomes.

Conclusion

Pediatric ON often leads to chronic demyelinating conditions. Visual recovery is overall good, but patients with NMOSD have worse visual outcomes and higher relapse rates.