探讨 2023 年 MOGAD 标准在有和无发作的 MOG 抗体阳性儿童和成人中的应用。
Investigating the 2023 MOGAD Criteria in Children and Adults With MOG-Antibody Positivity Within and Outside Attacks.
机构信息
From the Neuroimmunology Program (E.F., G.O.-C., E.M.-H., M.G., E.C., J.M.C.-M., S.L., Y.B., A.S., J.D., M.S., T.A.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic de Barcelona, Pediatric Neuroimmunology Unit (E.F., V.G.-Á., V.D., T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, and Sant Joan de Déu Private Foundation for Research and Education (IRSJD) Sant Joan de Déu Children's Hospital (E.F.), University of Barcelona; Pediatric Neurology Department (G.O.-C.), Hospital Parc Taulí de Sabadell; Immunology Service (L.N., R.R.G.), Hospital Clinic, and Ophtalmology Service (G.R., C.d.-P.-S.), Sant Joan de Déu Children's Hospital, University of Barcelona; Department of Neurology (J.B.-L.), University Hospital "12 de Octubre"; Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12) (J.B.-L.); Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED) (J.B.-L.); Department of Medicine, Faculty of Medicine (J.B.-L.), Complutense University, Madrid; Neurology Service (C.I.), Hospital Clínico Universitario, Zaragoza; Neurology Service (J.M.G.D.), Hospital General Universitario Gregorio Marañón, Madrid; Neurology Service (C.C.), Hospital Universitario Son Espases, Palma de Mallorca; Neurology Service (A.C.), Hospital de Mataró, Barcelona; Unit of Neuroimmunology and Multiple Sclerosis of Girona (UNIEMTG) (G.Á.B.), Department of Neurology, University Hospital Dr. Josep Trueta of Girona; Neurology Service (I.G.-S.), Hospital Álvaro Cunqueiro, Vigo; Department of Neurology (C.O.-G.), Hospital Clinico San Carlos, IdISSC, Madrid; Departament of Medicine (C.O.-G.), Medicine Faculty, Universidad Complutense de Madrid (UCM); Neurology Service (M.R.), Hospital Parc Taulí, Sabadell, Barcelona; Multiple Sclerosis CSUR and Clinical Neuroimmunology Unit (J.M.-P., J.E.M.-L.), Neurology Department, Hospital Clínico Universitario Virgen de la Arrixaca (IMIB-Arrixaca), Clinical Neuroimmunology and Multiple Sclerosis Cathedra, UCAM, Universidad Católica San Antonio de Murcia; Neurology Service (L.B.C.), Hospital Universitario Fundación Alcorcón, Madrid; Neurology Service (J.M.-M.), Hospital Universitario Miguel Servet, Zaragoza; Neurology Service (M.P., J.G.), Complejo Hospitalario Universitario de Albacete; Neurology Service (R.V.-G.), Hospital Universitario JM Morales Meseguer, Internal Medicine Department, Universidad de Murcia, Spain; Department of Neurology (J.D.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; and Catalan Institution for Research and Advanced Studies (ICREA) (J.D.), Barcelona, Spain.
出版信息
Neurology. 2024 Sep 24;103(6):e209682. doi: 10.1212/WNL.0000000000209682. Epub 2024 Aug 27.
BACKGROUND AND OBJECTIVES
The 2023 criteria for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) perform well in adults but have not been assessed in children.
METHODS
This prospective observational nationwide study includes children and adults with demyelinating syndromes or encephalitis, whose serum or CSF was found MOG-immunoglobulin G (IgG) positive at Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic of Barcelona (Spain). Exclusion criteria were lack of clinical information and follow-up <1 year, and serum unavailable for antibody testing. The primary outcome was to assess the accuracy of the 2023 MOGAD criteria, using as gold standard the most plausible diagnosis after a follow-up >1 year. MOGAD criteria were retrospectively applied assessing core syndromes, supportive clinical-radiological features, and MOG-IgG titers. Patients tested ≤3 months of a disease attack (acute phase) or afterward (remission) were considered separately. The positive predictive value (PPV) of the criteria (true-positive [patients classified as MOGAD and MOGAD diagnosis last follow-up] divided by total positive [all patients classified as MOGAD]), and its 95% CI, was calculated with the Wilson procedure.
RESULTS
A total of 257 patients (133 children) were included in the study (median age 15 years [interquartile range 6-38], 54% female). Among 202 patients assessed during a disease attack, 158 (78%) had high MOG-IgG serum titers, 36 (18%) low titers, and 8 (4%) antibodies only in CSF. No differences were identified between patients with high and low titers, but those with low titers were more likely to have an alternative diagnosis at last follow-up (2/36 [6%] vs 0/158, = 0.012). Supportive features were present in 230 of 257 (89%) patients, regardless of age, MOG-IgG titers, and core syndromes except for optic neuritis in adults whose assessment with orbital MRI was not systematic. Overall, 240 of 257 (94%) patients were well classified by the MOGAD criteria (e.g., 236 eventually having MOGAD and 4 alternative diagnoses), and 17 were wrongly classified (e.g., 11 eventually having MOGAD and 6 alternative diagnoses). Although the criteria classified better during disease attacks than during remissions (187 [96%] vs 49 [89%] serum MOG-IgG-positive patients were well-classified, = 0.038), the PPV was high in both settings (99% [95% CI 97-100] vs 98% [95% CI 89-100]).
DISCUSSION
The 2023 MOGAD criteria correctly identified most children and adults with MOGAD. The highest accuracy occurred when they were applied during disease attacks.
CLASSIFICATION OF EVIDENCE
This study provides Class IV evidence that the 2023 MOGAD criteria accurately identify adults and children with MOGAD.
背景与目的
2023 年髓鞘少突胶质细胞糖蛋白抗体相关性疾病（MOGAD）标准在成人中表现良好，但尚未在儿童中进行评估。
方法
本前瞻性观察性全国性研究纳入了在巴塞罗那的 Institut d'Investigacions Biomèdiques August Pi i Sunyer-Hospital Clínic 的脱髓鞘综合征或脑炎患者，其血清或 CSF 发现 MOG-免疫球蛋白 G（IgG）阳性。排除标准为缺乏临床信息和随访<1 年，以及血清无法进行抗体检测。主要结局是评估 2023 年 MOGAD 标准的准确性，将最有可能的诊断作为随访>1 年后的金标准。回顾性应用 MOGAD 标准评估核心综合征、支持性临床-放射学特征和 MOG-IgG 滴度。将在疾病发作后≤3 个月（急性期）或之后（缓解期）接受检测的患者分别考虑。使用 Wilson 程序计算标准的阳性预测值（真阳性[被分类为 MOGAD 且在最后一次随访中仍被诊断为 MOGAD 的患者]除以总阳性[所有被分类为 MOGAD 的患者]）及其 95%置信区间。
结果
共纳入 257 例患者（133 例为儿童）（中位年龄 15 岁[四分位距 6-38]，54%为女性）。在 202 例在疾病发作期间评估的患者中，158 例（78%）血清 MOG-IgG 滴度较高，36 例（18%）滴度较低，8 例（4%）仅在 CSF 中存在抗体。高滴度和低滴度患者之间未发现差异，但低滴度患者在最后一次随访时更有可能被诊断为其他疾病（2/36[6%]与 0/158，=0.012）。257 例患者中有 230 例（89%）存在支持性特征，与年龄、MOG-IgG 滴度和核心综合征无关，除了成人视神经炎，其评估不包括眼眶 MRI。总体而言，257 例患者中有 240 例（94%）被 MOGAD 标准很好地分类（例如，236 例最终被诊断为 MOGAD，4 例为其他诊断），17 例被错误分类（例如，11 例最终被诊断为 MOGAD，6 例为其他诊断）。虽然标准在疾病发作时比缓解期的分类效果更好（187 例[96%]与 49 例[89%]血清 MOG-IgG 阳性患者被很好地分类，=0.038），但阳性预测值在两种情况下均较高（99%[95%CI 97-100]与 98%[95%CI 89-100]）。
讨论
2023 年 MOGAD 标准正确识别了大多数患有 MOGAD 的儿童和成人。当在疾病发作期间应用时，准确性最高。
证据分类
本研究提供了 IV 级证据，表明 2023 年 MOGAD 标准可准确识别 MOGAD 的成人和儿童。
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