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Optic neuritis in Turkish children and adolescents: A multicenter retrospective study
Meltem Çobanoğulları Direk1, Şeyda Besen2, İbrahim Öncel3
1Department of Pediatrics, Division of Pediatric Neurology, Mersin University Faculty of Medicine, Faculty Of Medicine, 34, Cadde, Çiftlikköy Kampüsü, Mersin 33343, Türkiye.
Insights
Pediatric autoimmune optic neuritis presents differently by age, with older children more likely to develop multiple sclerosis. While most patients recover, relapsing forms and aquaporin-4 antibody-related optic neuritis indicate a less favorable prognosis.
Area of Science:
- Neurology
- Ophthalmology
- Immunology
Background:
- Autoimmune disorders are increasingly recognized causes of optic neuritis.
- Understanding demographic, clinical, and prognostic factors in pediatric autoimmune optic neuritis is crucial.
Purpose of the Study:
- To re-examine demographic, clinical, laboratory features, and prognostic factors in pediatric autoimmune optic neuritis.
- To update understanding based on current knowledge of autoantibody-mediated disorders.
Main Methods:
- Retrospective evaluation of pediatric optic neuritis cases from 27 centers in Türkiye (2009-2022).
- Analysis of 279 patients, categorized by age (prepubertal vs. others).
- Assessment of diagnoses, recurrences, treatments, and 12-month outcomes.
Main Results:
- Multiple sclerosis-associated optic neuritis was most common in older children (≥10 years).
- Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis was predominant in prepubertal children (<10 years).
- Recurrences occurred in 24% of patients, more frequently in females; 88.5% achieved complete recovery at 12 months.
Conclusions:
- Optic neuritis laterality differs by age: bilateral in prepubertal, unilateral in older children.
- Age ≥10, oligoclonal bands, and cranial MRI lesions predict multiple sclerosis development.
- Relapsing optic neuritis and AQP4-IgG-associated optic neuritis have poorer prognoses, requiring further research.
Background:
Various etiologies may underlie optic neuritis, including autoantibody-mediated disorders described in the last decade. We re-examined demographic, clinical, laboratory features and prognostic factors in pediatric patients with autoimmune optic neuritis according to current knowledge.
Methods:
Cases of pediatric ON from 27 centers in Türkiye diagnosed between 2009 and 2022 were included for retrospective evaluation.
Results:
The study included 279 patients, 174 females and 105 males, with a female-to-male ratio of 1.65. The average age at onset was 12.8 ± 3.4 years, and mean follow-up, 2.1 years (range: 1-12.1 years). Patients <10 years old were grouped as "prepubertal" and those ≥10 years old as "others". The diagnoses made at the end of follow-up were multiple sclerosis associated optic neuritis (n = 90, 32.3 %), single isolated optic neuritis (n = 86, 31 %), clinically isolated syndrome (n = 41, 14.7 %), myelin oligodendrocyte glycoprotein antibody associated optic neuritis (n = 22, 7.9 %), and relapsing isolated optic neuritis (n = 18, 6.5 %). Predominant diagnoses were myelin oligodendrocyte glycoprotein antibody associated optic neuritis and acute disseminated encephalomyelitis associated optic neuritis in the prepubertal group and multiple sclerosis associated optic neuritis in the older group. Recurrences were observed in 67 (24 %) patients, including 28 with multiple sclerosis associated optic neuritis, 18 with relapsing isolated optic neuritis, 11 with myelin oligodendrocyte glycoprotein antibody associated optic neuritis, 8 with aquaporin-4 antibody related optic neuritis, and 2 with chronic relapsing inflammatory optic neuropathy. Recurrences were more common among female patients. Findings supporting the diagnosis of multiple sclerosis included age of onset ≥ 10 years (OR=1.24, p = 0.027), the presence of cranial MRI lesions (OR=26.92, p<0.001), and oligoclonal bands (OR=9.7, p = 0.001). Treatment in the acute phase consisted of intravenous pulse methylprednisolone (n = 46, 16.5 %), pulse methylprednisolone with an oral taper (n = 212, 76 %), and combinations of pulse methylprednisolone, plasmapheresis, or intravenous immunoglobulin (n = 21, 7.5 %). Outcome at 12 months was satisfactory, with 247 out of 279 patients (88.5 %) demonstrating complete recovery. Thirty-two patients exhibited incomplete recovery and further combination treatments were applied. Specifically, patients with relapsing isolated optic neuritis and aquaporin-4 antibody related optic neuritis displayed a less favorable prognosis.
Conclusion:
Our results suggest optic neuritis is frequently bilateral in prepubertal and unilateral in peri‑ or postpubertal patients. Age of onset 10 or older, presence of oligoclonal bands, and brain MRI findings reliably predict the development of multiple sclerosis. The risk of developing multiple sclerosis increases mostly during the second and third years of follow-up. Relapsing isolated optic neuritis remains a separate group where the pathogenesis and outcome remain unclear. Investigation of predisposing and diagnostic biomarkers and long follow-up could help to define this group.
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