资源有限地区获得性脱髓鞘儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病诊断2023国际专家共识标准的验证与修正应用

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2025-08-19 DOI:10.1016/j.pediatrneurol.2025.08.010

Vishal Sondhi DM , Neelu Desai MD , Lokesh Lingappa DM , Vrajesh Udani MD , Naveen Sankhyan DM , Sheffali Gulati MD , Vykuntaraju K. Gowda DM , Lokesh Saini DM , Rachana Dubey DM , Ramesh Konanki DM , Jyotindra Narayan Goswami DM , Abhijeet Botre DNB , Pradnya Gadgil MRCPCH , Gouri Rao Passi MD , Anaita Udwadia Hegde MD , Mahesh Kamate DM , Abhishek Ravindra Jain MD , Jaya Shankar Kaushik DM , Suvasini Sharma DM , Kavita Srivastava MD , Ankit Kumar Meena DM

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引用次数: 0

摘要

背景：本研究旨在验证获得性脱髓鞘综合征儿童的2023髓鞘少突胶质细胞糖蛋白（MOG）抗体（Ab）相关疾病（MOGAD）诊断标准。方法：在这项多中心回顾性研究中，共有388名儿童符合2023年MOGAD标准。临床诊断MOGAD的190名儿童（真阳性），平均（IQR）年龄为7（4.7-10）岁，平均（IQR）随访时间为48（41-56）个月；196名儿童为真阴性，不符合2023年的标准或被诊断为MOGAD。两个孩子是假阳性，符合标准，但被诊断为多发性硬化症。2023 MOGAD诊断标准的灵敏度为100% (95% CI = 98.02%, 100%)，特异性为98.99% (95% CI = 96.39%, 99.82%)， PPV为98.96% (95% CI = 96.28%, 99.81%)， NPV为100% (95% CI = 98.08%, 100%)，诊断准确率为99.48% （95% CI = 98.15%, 99.94%）。MOGAD标准与单独使用MOG-Ab类似，因为所有MOGAD诊断仅限于血清阳性患者。结论：本研究表明2023 MOGAD诊断标准在获得性脱髓鞘儿童中表现优异；然而，该标准并没有提高单独抗体检测的诊断准确性。

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查看原文本刊更多论文

Validation and Modified Application of the 2023 International Expert Consensus Criteria for Diagnosing Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease Among Children With Acquired Demyelination in Resource-Limited Regions

Background

This study was undertaken to validate the 2023 myelin oligodendrocyte glycoprotein (MOG) antibody (Ab)-associated disease (MOGAD) diagnostic criteria among children with acquired demyelinating syndromes.

Methods

In this multicentric retrospective study, all children aged <18 years, diagnosed or followed up by pediatric neurologists for acquired demyelination between January 1, 2017, and December 31, 2022, were included if they had undergone at least one MOG-Ab testing. The 2023 MOGAD diagnostic criteria were applied retrospectively. The pediatric neurologist's clinical diagnosis of MOGAD served as the gold standard to assess the criteria's performance using sensitivity, specificity, PPV, and NPV.

Results

A total of 388 children were subjected to the 2023 MOGAD criteria. A clinical diagnosis of MOGAD was made in 190 children (true-positives), having a median (IQR) age of 7 (4.7-10) years and a median (IQR) follow-up of 48 (41-56) months; 196 children were true-negatives, not fulfilling 2023 criteria or diagnosed as MOGAD. Two children were false-positives, fulfilling criteria but diagnosed as multiple sclerosis. The 2023 MOGAD diagnostic criteria demonstrated a sensitivity of 100% (95% CI = 98.02%, 100%), specificity of 98.99% (95% CI = 96.39%, 99.82%), PPV of 98.96% (95% CI = 96.28%, 99.81%), NPV of 100% (95% CI = 98.08%, 100%), and diagnostic accuracy of 99.48% (95% CI = 98.15%, 99.94%). MOGAD criteria performed similar to MOG-Ab alone, as all MOGAD diagnoses were confined to seropositive patients.

Conclusion

The study demonstrates excellent performance of the 2023 MOGAD diagnostic criteria among children with acquired demyelination; however, the criteria did not enhance the diagnostic accuracy of antibody testing alone.

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来源期刊

Pediatric neurology 医学-临床神经学

CiteScore

4.80

自引率

2.60%

发文量

176

审稿时长

78 days

期刊介绍： Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.