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.
RodriguezMBrienPCOKurlandLT. Optic neuritis: a population-based study in Olrnsted County, Minnesota Neurology. 1995;45(2):244‐250.
3.
Langer-GouldAZhangJLChungJYeungYWaubantEYaoJ. Incidence of acquired CNS demyelinating syndromes in a multiethnic cohort of children.Neurology. 2011;77(12):1143-1148.
4.
BanwellBKennedyJSadovnickD, et al.Incidence of acquired demyelination of the CNS in Canadian children. Neurology. 2009;72(3):232‐239. doi:10.1212/01.wnl.0000339482.84392.bd
WilejtoMShroffMBuncicJRKennedyJGoiaCBanwellB. The clinical features, MRI findings, and outcome of optic neuritis in children. Neurology. 2006;67(2):258‐262. doi:10.1212/01.WNL.0000224757.69746.FB,
7.
PinelesSLRepkaMXLiuGT, et al.Assessment of pediatric optic neuritis visual acuity outcomes at 6 months. JAMA Ophthalmol. 2020;138(12):1253‐1261. doi:10.1001/JAMAOPHTHALMOL.2020.4231
8.
WanMJAdebonaOBensonLAGormanMPHeidaryG. Visual outcomes in pediatric optic neuritis. Am J Ophthalmol. 2014;158(3):503‐507.e2. doi:10.1016/J.AJO.2014.05.036
9.
ParkKAYangHKHanJ, et al.Characteristics of optic neuritis in South Korean children and adolescents: a retrospective multicenter study. J Ophthalmol. 2022;2:1‐8. doi:10.1155/2022/4281772
10.
WaldmanATStullLBGalettaSLBalcerLJLiuGT. Pediatric optic neuritis and risk of multiple sclerosis: meta-analysis of observational studies. J Am Assoc Pediatr Ophthalmol Strabismus. 2011;15(5):441‐446. doi:10.1016/J.JAAPOS.2011.05.020
KunchokAChenJJMcKeonAMillsJRFlanaganEPPittockSJ. Coexistence of myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies in adult and pediatric patients. JAMA Neurol. 2020;77(2):257‐259. doi:10.1001/jamaneurol.2019.3656
13.
KruppLBBanwellBTenembaumS. Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 2007;68(16 suppl 2):S7-S12. doi:10.1212/01.WNL.0000259422.44235.A8
14.
WingerchukDMBanwellBBennettJL, et al.International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177‐189. doi:10.1212/WNL.0000000000001729
15.
BanwellBBennettJLMarignierR, et al.Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol. 2023;22(3):268‐282. doi:10.1016/S1474-4422(22)00431-8
16.
TomaiuoloMWoretaFALiA, et al.Open-globe injury repairs in the American Academy of Ophthalmology IRIS® Registry 2014-2018: Incidence, risk factors, and visual outcomes. Ophthalmology. 2023;130(8):812‐821. doi:10.1016/j.ophtha.2023.03.002
17.
GaudiosoCMMarSCasperTC, et al.MOG And AQP4 antibodies among children with multiple sclerosis and controls. Ann Neurol. 2023;93(2):271‐284. doi:10.1002/ANA.26502
BonhommeGRWaldmanATBalcerLJ, et al.Pediatric optic neuritis: brain MRI abnormalities and risk of multiple sclerosis. Neurology. 2009;72(10):881‐885. doi:10.1212/01.WNL.0000344163.65326.48
20.
SongHZhouHYangM, et al.Clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein antibody-seropositive paediatric optic neuritis in China. Br J Ophthalmol. 2019;103(6):831‐836. doi:10.1136/BJOPHTHALMOL-2018-312399
21.
LeeJYHanJYangMOhSY. Population-based incidence of pediatric and adult optic neuritis and the risk of multiple sclerosis. Ophthalmology. 2020;127(3):417‐425. doi:10.1016/J.OPHTHA.2019.09.032
22.
SudhakarPKiniA. Clinical features and visual outcome of pediatric optic neuritis. Indian J Ophthalmol. 2023;71(6):2603‐2604. doi:10.4103/IJO.IJO_440_23
23.
HeussingerNKontopantelisERompelOPaulidesMTrollmannR. Predicting multiple sclerosis following isolated optic neuritis in children. Eur J Neurol. 2013;20(9):1292‐1296. doi:10.1111/ENE.12184
24.
CorteseRCarrascoFPTurC, et al.Differentiating multiple sclerosis from AQP4-neuromyelitis Optica spectrum disorder and MOG-antibody disease with imaging. Neurol ®. 2023;100(3):308‐323. doi:10.1212/WNL.0000000000201465
25.
PittockSJLennonVAKreckeKWingerchukDMLucchinettiCFWeinshenkerBG. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63(3):390‐396. doi:10.1001/ARCHNEUR.63.3.390,
26.
KimHJPaulFLana-PeixotoMA, et al.MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 2015;84(11):1165‐1173. doi:10.1212/WNL.0000000000001367
27.
MontalbanXLebrun-FrénayCOhJ, et al.Diagnosis of multiple sclerosis: 2024 revisions of the McDonald criteria. Lancet Neurol. 2025;24(10):850‐865. doi:10.1016/S1474-4422(25)00270-4
28.
BeckRWClearyPAAndersonMM, et al.A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med. 1992;326(9):581‐588. doi:10.1056/NEJM199202273260901
29.
BeckRWMokePSTrobeJD. The 5-year risk of MS after optic neuritis. Neurology. 1998;51(4):1237‐1238. doi:10.1212/wnl.51.4.1237-a
30.
GalRLBrodskyMNazarianS, et al.Visual function 15 years after optic neuritis. A final follow-up report from the Optic Neuritis Treatment Trial. Ophthalmology. 2008;115(6):1082.e5. doi:10.1016/j.ophtha.2007.08.004
31.
MinYGMoonYKwonYN, et al.Prognostic factors of first-onset optic neuritis based on diagnostic criteria and antibody status: a multicentre analysis of 427 eyes. J Neurol Neurosurg Psychiatry. 2024;95(8):753‐760. doi:10.1136/jnnp-2023-333133
32.
Wilf-YarkoniAFeldmannKRubarthK, et al.Effectiveness of oral prednisone tapering following intravenous methylprednisolone for acute optic neuritis in multiple sclerosis. PLoS One. 2023;18(12):1‐11. doi:10.1371/journal.pone.0288366
33.
RoedHGLangkildeASellebjergF, et al.A double-blind, randomized trial of IV immunoglobulin treatment in acute optic neuritis. Neurology. 2005;64(5):804‐810. doi:10.1212/01.WNL.0000152873.82631.B3,
34.
TselisAPerumalJCaonC, et al.Treatment of corticosteroid refractory optic neuritis in multiple sclerosis patients with intravenous immunoglobulin. Eur J Neurol. 2008;15(11):1163‐1167. doi:10.1111/J.1468-1331.2008.02258.X,
35.
BigiSBanwellBYehEA. Outcomes after early administration of plasma exchange in pediatric central nervous system inflammatory demyelination. J Child Neurol. 2015;30(7):874‐880. doi:10.1177/0883073814545883,
36.
KoziolekMMühlhausenJFriedeT, et al.Therapeutic apheresis in pediatric patients with acute CNS inflammatory demyelinating disease. Blood Purif. 2013;36(2):92‐97. doi:10.1159/000354077,
37.
ChenQZhaoGHuangY, et al.Clinical characteristics of pediatric optic neuritis with myelin oligodendrocyte glycoprotein seropositive: a cohort study. Pediatr Neurol. 2018;83:42‐49. doi:10.1016/j.pediatrneurol.2018.03.003
