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Peer-reviewedCC BYAnnals of medicine

External validation of the MOG-AR score in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease.

4 affiliations
  1. 1Department of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
  2. 2Department of Neurology, The Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
  3. 3Department of Radiology, The Affiliated Children's Hospital of Xiangya School of Medicine, Central South University (Hunan Children's Hospital), Changsha, China.
  4. 4Pediatrics Research Institute, Hunan Children's Hospital, Central South University, Changsha, Hunan, China.

Abstract

Background

Predicting the risk of relapse in myelin oligodendrocyte glycoprotein antibody-associated disease is essential for guiding early treatment decisions. A score evaluating onset age, sex, attack phenotype, and treatment received (MOG-AR) has been proposed to identify patients at high relapse risk since disease onset, but has never been validated in a pediatric population. This study aimed to externally validate the MOG-AR score in an exclusively pediatric cohort.Patients and methods

We retrospectively identified 139 children with myelin oligodendrocyte glycoprotein antibody-associated disease (age <18 years) within the past decade. The MOG-AR score was calculated and the patients were stratified into four risk grades. Discrimination and calibration were assessed using multiple complementary metrics, and univariable analyses were performed for each score component. Sensitivity analyses included exploratory reclassification using modified MOG-AR grade thresholds and the development of an exploratory phenotype-based model.Results

We included 139 children with a median follow-up of 43 months; relapse occurred in 28.8% of patients. Harrell's concordance index was 0.472 (95% confidence interval, 0.366-0.577), and the time-dependent area under the receiver operating characteristic curve ranged from 0.467 to 0.510 over 1-5 years. Calibration assessment indicated limited variation in predicted probabilities across risk grades. Modified MOG-AR grade thresholds did not improve risk group separation (log-rank p = 0.359) or alter continuous scores or rankings. The exploratory phenotype-based model showed group separation on log-rank testing (p = 0.031) but poor discrimination (C-index, 0.394).Conclusions

In this single-center pediatric cohort, the MOG-AR score did not provide clinically useful stratification of first-relapse risk. Limited variability in the age and treatment-related components may have contributed to its limited performance. Further multicenter validation in pediatric cohorts with broader case-mix and treatment patterns is warranted. The exploratory phenotype-based model requires independent prospective validation.

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BibTeX
@article{xiao2026external,
  title = {{External validation of the MOG-AR score in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease.}},
  author = {Sha Xiao and Li Li and Zhimin Zou and Yongjie Liu and Longlong Xie and Kuikui Zeng and Maxuan Hu and Wenliang Yang and Sai Yang and Lingjuan Liu},
  journal = {Annals of medicine},
  year = {2026},
  month = oct,
  doi = {10.1080/07853890.2026.2735072},
  url = {https://europepmc.org/article/MED/42895452},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - External validation of the MOG-AR score in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease.
AU  - Sha Xiao
AU  - Li Li
AU  - Zhimin Zou
AU  - Yongjie Liu
AU  - Longlong Xie
AU  - Kuikui Zeng
AU  - Maxuan Hu
AU  - Wenliang Yang
AU  - Sai Yang
AU  - Lingjuan Liu
PY  - 2026
DA  - 2026/10/19
JO  - Annals of medicine
DO  - 10.1080/07853890.2026.2735072
UR  - https://europepmc.org/article/MED/42895452
AB  - Background  Predicting the risk of relapse in myelin oligodendrocyte glycoprotein antibody-associated disease is essential for guiding early treatment decisions. A score evaluating onset age, sex, attack phenotype, and treatment received (MOG-AR) has been proposed to identify patients at high relapse risk since disease onset, but has never been validated in a pediatric population. This study aimed to externally validate the MOG-AR score in an exclusively pediatric cohort.Patients and methods  We retrospectively identified 139 children with myelin oligodendrocyte glycoprotein antibody-associated disease (age <18 years) within the past decade. The MOG-AR score was calculated and the patients were stratified into four risk grades. Discrimination and calibration were assessed using multiple complementary metrics, and univariable analyses were performed for each score component. Sensitivity analyses included exploratory reclassification using modified MOG-AR grade thresholds and the development of an exploratory phenotype-based model.Results  We included 139 children with a median follow-up of 43 months; relapse occurred in 28.8% of patients. Harrell's concordance index was 0.472 (95% confidence interval, 0.366-0.577), and the time-dependent area under the receiver operating characteristic curve ranged from 0.467 to 0.510 over 1-5 years. Calibration assessment indicated limited variation in predicted probabilities across risk grades. Modified MOG-AR grade thresholds did not improve risk group separation (log-rank p = 0.359) or alter continuous scores or rankings. The exploratory phenotype-based model showed group separation on log-rank testing (p = 0.031) but poor discrimination (C-index, 0.394).Conclusions  In this single-center pediatric cohort, the MOG-AR score did not provide clinically useful stratification of first-relapse risk. Limited variability in the age and treatment-related components may have contributed to its limited performance. Further multicenter validation in pediatric cohorts with broader case-mix and treatment patterns is warranted. The exploratory phenotype-based model requires independent prospective validation.
SN  - 0785-3890
SN  - 1365-2060
ER  -