Fonseca Elianet Gisell, Olivé-Cirera Gemma, Chen Li-Wen, Paredes-Carmona Fernando, Nuñez Enamorado Noemí, Boyero Duran Sabas, Mendibe Maria Del Mar, Visa-Reñé Núria, Vázquez-López María, Felipe-Rucián Ana, Romeu Gemma, Martinez-Hernandez Eugenia, Blanco Yolanda, Sepulveda Maria, Saiz Albert, Dalmau Josep, Armangue Thaís
Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) - CaixaResearch Institute, Hospital Clínic de Barcelona, Barcelona, Spain.
Pediatric Neuroimmunology Unit, Neurology Department, Sant Joan de Déu Children's Hospital, ERN-RITA reference center, University of Barcelona, Barcelona, Spain.
Neurol Neuroimmunol Neuroinflamm. 2025 Jul;12(4):e200400. doi: 10.1212/NXI.0000000000200400. Epub 2025 Jun 12.
The aim of this study was to report the clinical features, disease-modifying treatment (DMT) response, and outcomes of children with MOG-IgG who fulfill the 2017 McDonald criteria for multiple sclerosis (MS).
This prospective observational study included children (<18 years) with a suspected acquired demyelinating syndrome (ADS) whose serum or CSF was positive for MOG-IgG, who met the indicated MS criteria, and who had ≥1 year of clinical follow-up. MOG-IgG was tested using live cell-based assays.
Of 554 children with confirmed ADS (196 with MOG-IgG), 8 (median age 11 years, interquartile range 9-14) harbored MOG-IgG and fulfilled MS criteria: 2 had typical MS and 6 had overlapping MOGAD-MS features at onset, but 5 of the latter group developed an MS-like course during follow-up. Five of 7 patients with assessable samples were Epstein-Barr virus seropositive at disease onset, and all 8 had persistent silent radiologic activity with lesional location and morphology suggestive of MS, leading to initiation of DMT. All initial treatments were well tolerated, but eventually, 7 of 8 children (88%) required high-efficacy DMT.
In this pediatric cohort, 4% of patients with MOG-IgG met criteria for MS. The clinical-radiologic spectrum ranged from typical MS to overlapping MOGAD-MS, and patients usually required high-efficacy DMT.
本研究旨在报告符合2017年多发性硬化症（MS）麦克唐纳标准的MOG-IgG阳性儿童的临床特征、疾病修饰治疗（DMT）反应及预后。
这项前瞻性观察性研究纳入了年龄小于18岁、疑似获得性脱髓鞘综合征（ADS）、血清或脑脊液MOG-IgG阳性、符合指定MS标准且有≥1年临床随访的儿童。采用基于活细胞的检测方法检测MOG-IgG。
在554例确诊为ADS的儿童（196例MOG-IgG阳性）中，8例（中位年龄11岁，四分位间距9-14岁）MOG-IgG阳性且符合MS标准：2例为典型MS，6例起病时有重叠的MOGAD-MS特征，但后一组中有5例在随访期间发展为类似MS的病程。7例有可评估样本的患者中有5例在疾病发作时EB病毒血清学阳性，所有8例均有持续的无症状放射学活动，病变部位和形态提示为MS，因此开始使用DMT。所有初始治疗耐受性良好，但最终，8名儿童中有7名（88%）需要高效DMT。
在这个儿科队列中，4%的MOG-IgG阳性患者符合MS标准。临床-放射学谱范围从典型MS到重叠的MOGAD-MS，患者通常需要高效DMT。
