Evaluating the Clinical Characteristics and Ophthalmic Manifestations in Children and Adolescents with Acquired Demyelinating Disorders in China
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Updated: May 10, 2026
Dynamic Visual Tests to Identify and Quantify Visual Damage and Repair Following Demyelination in Optic Neuritis Patients
Published on: April 14, 2014
Si-Yuan Li1, Chun-Xia Peng1, Hua-Xin Zuo1
1Department of Ophthalmology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Insights
Pediatric acquired demyelinating disorders in Chinese children often present earlier and affect both eyes. Neuromyelitis optica spectrum disorder patients had worse vision and thinner retinal nerve fiber layers but improved with methylprednisolone.
Area of Science:
- Neurology
- Ophthalmology
- Immunology
Background:
- Pediatric acquired demyelinating disorders (ADS) encompass inflammatory central nervous system (CNS) conditions.
- Optic neuritis (ON) is a key ADS manifestation involving autoimmune inflammation of optic nerve axons.
Purpose of the Study:
- To investigate clinical characteristics, ophthalmic findings, laboratory results, and prognostic factors in Chinese pediatric ADS patients.
- To analyze demographic data, visual acuity, antibody serostatus, and treatment outcomes.
Main Methods:
- Retrospective analysis of clinical data from 57 pediatric ADS patients (March 2021-December 2023).
- Evaluated best-corrected visual acuity (BCVA), peripapillary retinal nerve fiber layer (RNFL) thickness, and serum MOG/AQP4 antibody levels.
- Assessed outcomes after intravenous methylprednisolone therapy.
Main Results:
- The study included 38 ON, 12 NMOSD, 5 ADEM, and 2 MS patients; median ON onset age was 9 years, with 40.4% ≤8 years and 64.9% bilateral involvement.
- MOG-antibody positivity was 50%, and AQP4-antibody positivity was 19.4%.
- NMOSD patients showed significantly worse visual acuity (84.2% low vision) and thinner RNFL compared to ON patients (p < 0.05).
- Affected eyes had thinner RNFL than unaffected eyes (median 72.0 μm vs. 102.0 μm, p < 0.05).
- Both ON and NMOSD groups demonstrated good visual recovery (BCVA ≥0.4) after methylprednisolone treatment.
Conclusions:
- Chinese pediatric ADS patients exhibit distinct features: earlier onset and more frequent bilateral involvement.
- NMOSD in this population is associated with severe visual impairment and reduced RNFL thickness.
- Methylprednisolone is effective for visual recovery in pediatric ON and NMOSD.
Abstract:
Aims/Background Pediatric acquired demyelinating disorders (ADS) include various monophasic and recurrent inflammatory conditions of the central nervous system (CNS). Optic neuritis (ON) is a demyelinating disease primarily affecting the optic nerve axons due to autoimmune inflammation. To investigate the clinical characteristics, ophthalmic manifestations, laboratory test results, and prognostic indicators of Chinese children and adolescents with ADS. Methods The clinical data of 57 patients with ADS treated in the Beijing Children's Hospital from March 2021 to December 2023, were retrospectively collected and analyzed. The primary outcomes include best-corrected visual acuity (BCVA) at onset, peripapillary retinal nerve fiber layers (RNFL) thickness, serum myelin oligodendrocyte glycoprotein (MOG) antibody level, aquaporin-4 (AQP4) antibody level, and final BCVA after hormone therapy. Results The analyses included 57 children and adolescents in the study, including 38 optic neuritis (ON), 12 neuromyelitis optica spectrum disorder (NMOSD), 5 acute disseminated encephalomyelitis (ADEM), and 2 multiple sclerosis (MS) patients. The median age of patients facing the initial attack of ON was 9 years (3-15 years), with 23 children and adolescents (40.4%) aged eight years or younger, and 37 patients (64.9%) were bilaterally affected. Half of the children and adolescents (15/30) were MOG-Ab seropositive, and 19.4% (6/31) were AQP4-Ab seropositive. Children and adolescents with NMOSD were more likely to have severe visual impairment at acute onset (p < 0.05), with 84.2% of them having low vision acuity (BCVA worse than 0.1), as compared with 64.9% in the ON group. The RNFL thickness of affected eye was thinner than unaffected eyes (median 72.0 μm vs. median 102.0 μm, p < 0.05). Patients with NMOSD exhibited lower RNFL thickness across all measured sectors compared to those with ON. Significant differences were noted in the average RNFL and in each specific region of the retina, including the temporal RNFL, nasal RNFL, nasal superior RNFL, nasal inferior RNFL, temporal superior RNFL, and temporal inferior RNFL (all p < 0.05). After the treatment with intravenous methylprednisolone, both patients with ON (52 eyes; 86.7%) or NMOSD (20 eyes; 90.9%) group had functional visual recovery (BCVA better or equal 0.4). Conclusion This study shows that Chinese children and adolescents with ADS present distinctive clinical features, including earlier onset and more bilateral involvement. Furthermore, NMOSD patients experience more severe visual acuity impairment and thinner peripapillary RNFL. All children and adolescents of ON or NMOSD respond well to methylprednisolone treatment.
