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Dynamic Visual Tests to Identify and Quantify Visual Damage and Repair Following Demyelination in Optic Neuritis Patients
Published on: April 14, 2014
Outcomes of Children With Optic Neuritis as Their First Demyelinating Event
Lydia Marcus1, Andrew Van1, Tanya Correya2
1The University of Alabama at Birmingham, Birmingham, AL, USA.
Insights
Pediatric optic neuritis (ON) can lead to chronic demyelinating conditions. While visual recovery is generally good, children with aquaporin-4 receptor antibody disease (NMOSD) experience poorer outcomes and more relapses.
Area of Science:
- Neurology
- Ophthalmology
- Immunology
Background:
- Optic neuritis (ON) is a common cause of acute optic nerve inflammation in children and adults.
- This study focuses on pediatric patients experiencing their first ON episode.
- Understanding the diverse causes and outcomes of pediatric ON is crucial for effective management.
Purpose of the Study:
- To describe the clinical and diagnostic features of pediatric ON.
- To evaluate visual outcomes and relapse risks in children with ON.
- To differentiate outcomes based on specific antibody statuses and diagnoses.
Main Methods:
- Prospective identification of 50 children with their first ON episode.
- Serum antibody screening for myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4).
- Analysis of demographics, MRI findings, treatments, relapses, and visual disability.
Main Results:
- Patients were stratified into idiopathic ON, MOG antibody disease, multiple sclerosis, and NMOSD.
- Visual acuity loss was nearly universal (96%) at onset.
- Neuromyelitis optica spectrum disorder (NMOSD) patients showed the most severe vision loss and highest relapse rates.
- Half of the cohort experienced relapses, with ON and transverse myelitis being most common.
Conclusions:
- Pediatric ON frequently results in chronic demyelinating conditions.
- Overall visual recovery is positive, but NMOSD cases present with worse visual outcomes and higher relapse frequency.
- Early diagnosis and appropriate management are essential for improving long-term prognoses in pediatric ON.
Abstract:
IntroductionOptic 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.MethodsWe 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.ResultsSubjects 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.ConclusionPediatric ON often leads to chronic demyelinating conditions. Visual recovery is overall good, but patients with NMOSD have worse visual outcomes and higher relapse rates.

