Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients

Abstract Background The clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have been found to overlap with several other diseases. The new criteria proposed in 2023 were designed to better identify the disease but require validation across various populati...

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Main Authors: Meng-Ting Cai, Yi Hua, Qi-Lun Lai, Sheng-Yao Su, Chun-Hong Shen, Song Qiao, Yong-Feng Xu, Zhe-Feng Yuan, Yin-Xi Zhang
Format: Article
Language:English
Published: BMC 2025-01-01
Series:BMC Medicine
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Online Access:https://doi.org/10.1186/s12916-025-03875-9
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author Meng-Ting Cai
Yi Hua
Qi-Lun Lai
Sheng-Yao Su
Chun-Hong Shen
Song Qiao
Yong-Feng Xu
Zhe-Feng Yuan
Yin-Xi Zhang
author_facet Meng-Ting Cai
Yi Hua
Qi-Lun Lai
Sheng-Yao Su
Chun-Hong Shen
Song Qiao
Yong-Feng Xu
Zhe-Feng Yuan
Yin-Xi Zhang
author_sort Meng-Ting Cai
collection DOAJ
description Abstract Background The clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have been found to overlap with several other diseases. The new criteria proposed in 2023 were designed to better identify the disease but require validation across various populations to ascertain its clinical utility. We aimed to investigate the diagnostic performance in phenotypically diverse patients. Methods This multicenter study retrospectively included adult and pediatric patients who were hospitalized for a first suspected demyelinating event and tested positive for MOG immunoglobulin G (IgG) during the acute phase. The 2023 Lancet Neurology criteria were assessed against the benchmark of empirical clinical diagnosis, and the 2018 JAMA Neurology and Journal of Neuroinflammation criteria were also evaluated for comparative analysis. Results Among the 291 eligible patients (82 adults, 209 children), 282 (96.9%) were clinically diagnosed as definite MOGAD (77 adults, 205 children), while 262 (90.0%) fulfilled the 2023 diagnostic criteria (78 adults, 184 children). A total of 265 patients met the criteria for core clinical demyelinating events, and 76 (26.1%) had serum clear positive MOG-IgG (≥ 1:100). The sensitivity of the 2023 criteria was 0.91 (adults vs. children = 0.97 vs. 0.89), the specificity was 0.56 (adults vs. children = 0.40 vs. 0.75), positive likelihood ratio was 2.06 (adults vs. children = 1.62 vs. 3.57), and negative likelihood ratio (NLR) was 0.15 (adults vs. children = 0.06 vs. 0.14). Additionally, 264 and 256 cases were classified as definite MOGAD by the 2018 JAMA Neurology and Journal of Neuroinflammation criteria, respectively. Compared to the 2023 diagnostic criteria, the 2018 JAMA Neurology criteria demonstrated similar diagnostic performance. However, the 2018 Journal of Neuroinflammation criteria exhibited comparable sensitivity (0.92, adults vs. children = 0.96 vs. 0.89), higher specificity (1.00, adults vs. children = 1.00 vs. 1.00) and better NLR (0.09, adults vs. children = 0.04 vs. 0.11). Conclusions The 2023 criteria demonstrated good sensitivity in adult and pediatric patients in China yet modest specificity. Close follow-up is needed for patients with atypical phenotypes but high-titer MOG-IgG to avoid underdiagnosis.
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spelling doaj-art-1c69b0c0448c4c03bbd01c37fa403b962025-01-26T12:37:13ZengBMCBMC Medicine1741-70152025-01-0123111410.1186/s12916-025-03875-9Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patientsMeng-Ting Cai0Yi Hua1Qi-Lun Lai2Sheng-Yao Su3Chun-Hong Shen4Song Qiao5Yong-Feng Xu6Zhe-Feng Yuan7Yin-Xi Zhang8Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang UniversityDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthDepartment of Neurology, Zhejiang HospitalDepartment of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang UniversityDepartment of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang UniversityDepartment of Neurology, Zhejiang HospitalDepartment of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang UniversityDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child HealthDepartment of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang UniversityAbstract Background The clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have been found to overlap with several other diseases. The new criteria proposed in 2023 were designed to better identify the disease but require validation across various populations to ascertain its clinical utility. We aimed to investigate the diagnostic performance in phenotypically diverse patients. Methods This multicenter study retrospectively included adult and pediatric patients who were hospitalized for a first suspected demyelinating event and tested positive for MOG immunoglobulin G (IgG) during the acute phase. The 2023 Lancet Neurology criteria were assessed against the benchmark of empirical clinical diagnosis, and the 2018 JAMA Neurology and Journal of Neuroinflammation criteria were also evaluated for comparative analysis. Results Among the 291 eligible patients (82 adults, 209 children), 282 (96.9%) were clinically diagnosed as definite MOGAD (77 adults, 205 children), while 262 (90.0%) fulfilled the 2023 diagnostic criteria (78 adults, 184 children). A total of 265 patients met the criteria for core clinical demyelinating events, and 76 (26.1%) had serum clear positive MOG-IgG (≥ 1:100). The sensitivity of the 2023 criteria was 0.91 (adults vs. children = 0.97 vs. 0.89), the specificity was 0.56 (adults vs. children = 0.40 vs. 0.75), positive likelihood ratio was 2.06 (adults vs. children = 1.62 vs. 3.57), and negative likelihood ratio (NLR) was 0.15 (adults vs. children = 0.06 vs. 0.14). Additionally, 264 and 256 cases were classified as definite MOGAD by the 2018 JAMA Neurology and Journal of Neuroinflammation criteria, respectively. Compared to the 2023 diagnostic criteria, the 2018 JAMA Neurology criteria demonstrated similar diagnostic performance. However, the 2018 Journal of Neuroinflammation criteria exhibited comparable sensitivity (0.92, adults vs. children = 0.96 vs. 0.89), higher specificity (1.00, adults vs. children = 1.00 vs. 1.00) and better NLR (0.09, adults vs. children = 0.04 vs. 0.11). Conclusions The 2023 criteria demonstrated good sensitivity in adult and pediatric patients in China yet modest specificity. Close follow-up is needed for patients with atypical phenotypes but high-titer MOG-IgG to avoid underdiagnosis.https://doi.org/10.1186/s12916-025-03875-9Myelin oligodendrocyte glycoprotein antibody-associated disease2023 diagnostic criteriaCore demyelinating eventAntibody titerReal-world applicationMulticenter cohort
spellingShingle Meng-Ting Cai
Yi Hua
Qi-Lun Lai
Sheng-Yao Su
Chun-Hong Shen
Song Qiao
Yong-Feng Xu
Zhe-Feng Yuan
Yin-Xi Zhang
Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
BMC Medicine
Myelin oligodendrocyte glycoprotein antibody-associated disease
2023 diagnostic criteria
Core demyelinating event
Antibody titer
Real-world application
Multicenter cohort
title Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
title_full Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
title_fullStr Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
title_full_unstemmed Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
title_short Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients
title_sort performance of the 2023 diagnostic criteria for mogad real world application in a chinese multicenter cohort of pediatric and adult patients
topic Myelin oligodendrocyte glycoprotein antibody-associated disease
2023 diagnostic criteria
Core demyelinating event
Antibody titer
Real-world application
Multicenter cohort
url https://doi.org/10.1186/s12916-025-03875-9
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