Assessment of Pediatric Optic Neuritis Visual Acuity Outcomes at 6 Months

Abstract

IMPORTANCE Optic neuritis (ON) in children is uncommon. There are limited prospective data for visual acuity (VA) outcomes, associated diseases, and neuroimaging findings. Prospective data from a large sample would be useful for counseling families on treatment decisions and prognosis.OBJECTIVE To prospectively study children with a first episode of ON, describe VA after 6 months, and ascertain the network's (Pediatric Eye Disease Investigator Group and Neuro-Ophthalmology Research Disease Investigator Consortium) ability to enroll pediatric patients with ON prospectively. DESIGN, SETTING, AND PARTICIPANTSThis nonrandomized cohort study was conducted from September 20, 2016, to July 20, 2018, at 23 sites in the United States and Canada in pediatric ophthalmology or neuro-ophthalmology clinics. A total of 44 children (aged 3-15 years) presented with a first episode of ON (visual loss, pain on eye movements, or both) within 2 weeks of symptom onset and at least 1 of the following in the affected eye: a distance high-contrast VA (HCVA) deficit of at least 0.2 logMAR below age-based norms, diminished color vision, abnormal visual field, or optic disc swelling. Exclusion criteria included preexisting ocular abnormalities or a previous episode of ON.MAIN OUTCOMES AND MEASURES Primary outcomes were monocular HCVA and low-contrast VA at 6 months. Secondary outcomes were neuroimaging, associated diagnoses, and antibodies for neuromyelitis optica and myelin oligodendrocyte glycoprotein.RESULTS A total of 44 children (mean age [SD], 10.2 [3.5] years; 26 boys [59%]; 23 White individuals [52%]; 54 eyes) were enrolled in the study. Sixteen patients (36%) had bilateral ON. Magnetic resonance imaging revealed white matter lesions in 23 children (52%). Of these children, 8 had myelin oligodendrocyte glycoprotein-associated demyelination (18%), 7 had acute disseminated encephalomyelitis (16%), 5 had multiple sclerosis (11%), and 3 had neuromyelitis optica (7%). The baseline mean HCVA was 0.95 logMAR (20/200), which improved by a mean 0.76 logMAR (95% CI, 0.54-0.99; range, -0.70 to 1.80) to 0.12 logMAR (20/25) at 6 months. The baseline mean distance low-contrast VA was 1.49 logMAR (20/640) and improved by a mean 0.72 logMAR (95% CI, 0.54-0.89; range, -0.20 to 1.50) to 0.73 logMAR (20/100) at 6 months. Baseline HCVA was worse in younger participants (aged <10 years) with associated neurologic autoimmune diagnoses, white matter lesions, and in those of non-White race and non-Hispanic ethnicity. The data did not suggest a statistically significant association between baseline factors and improvement in HCVA. CONCLUSIONS AND RELEVANCEThe study network did not reach its targeted enrollment of 100 pediatric patients with ON over 2 years. This indicates that future treatment trials may need to use different inclusion criteria or plan a longer enrollment period to account for the rarity of the disease. Despite poor VA at presentation, most children had marked improvement by 6 months. Associated neurologic autoimmune diagnoses were common. ...

Working up a child with demyelinating optic neuritis: Striking a balance!

“…best-corrected visual acuity <20/200 at presentation. [ 5 6 ] However, pain on eye movements is relatively infrequent in PON as compared to adults,[ 5 6 ] although it matches the reported prevalence from the southeast Asia. [ 4 ] These differences are particularly marked in younger children, however, optic neuritis in adolescents tends to resemble AON.…”

“…[ 5 ] They reported that at 6-month follow-up, based on the clinical presentation, magnetic resonance imaging (MRI) findings, and serological testing, the final diagnosis in this study was: Isolated optic neuritis in 48%, MOG associated optic neuritis in 18%, MS in 11%, ADEM in 16%, and NMOSD in 7%. [ 5 ] Further, in this study, they could reclassify four patients with idiopathic ON (9%), 1 (2%) patient with ADEM, and 2 (5%) with seronegative NMO as MOG-positive ON,[ 5 ] highlighting that MOG optic neuritis is one of the predominant etiologies in this age group, and serological testing for MOG should be part of the routine workup in these patients. However, this study could not provide clues to specific neuro-inflammatory etiologies.…”

“…While optic neuritis especially in younger children tends to be bilateral and manifests as papillitis, unilateral retrobulbar neuritis is more common in the adults. [ 3 4 5 ] Children with optic neuritis often present with profound vision loss, i.e. best-corrected visual acuity <20/200 at presentation.…”

“…Common neuro-inflammatory syndromes that cause demyelinating optic neuritis in children include multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD), or Myelin-oligodendrocyte glycoprotein (MOG) associated optic neuritis. [ 4 5 7 8 9 ] Following the widespread availability of MOG antibody testing, studies have shown a high frequency of positivity for MOG antibodies in children presenting with optic neuritis. [ 5 7 ]…”

“…[ 4 5 7 8 9 ] Following the widespread availability of MOG antibody testing, studies have shown a high frequency of positivity for MOG antibodies in children presenting with optic neuritis. [ 5 7 ]…”