Exploring Treatment Approaches in Pediatric MOG Antibody-Associated Disease: A Survey of Neurologists.

IF 2.3 Q3 CLINICAL NEUROLOGY
Neurology. Clinical practice Pub Date : 2025-08-01 Epub Date: 2025-06-27 DOI:10.1212/CPJ.0000000000200504
Rabporn Suntornlohanakul, Carmen Yea, E Ann Yeh
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引用次数: 0

Abstract

Background and objectives: Diagnostic criteria for anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases (MOGADs) were published in 2023, but debate regarding optimal therapeutic strategies for pediatric MOGAD exists. The aim of this study was to evaluate treatment approaches and preferred diagnostic investigations for pediatric MOGAD among neurologists.

Methods: A survey questionnaire focused on pediatric MOGAD treatment was launched through the Practice Current Section of Neurology® Clinical Practice in April-October 2024. Responses from neurologists were solicited through advertisements on American Academy of Neurology (AAN) social media platforms, on the AAN website, and in print editions of Neurology®, as well as through QR codes shared at professional neurologic meetings. The questionnaire included 12 questions evaluating clinical decision making after a first and second neuroinflammatory episode, in a child testing positive for MOG-IgG antibody. Demographic questions were included. Responses were evaluated using descriptive statistics. A comparative analysis was conducted between those who self-identified as neuroimmunologists (NIs) and those who did not.

Results: A total of 346 neurologists completed the survey (52.3% of general neurologists, 32.1% of NIs, and 15.6% in other neurology fields). Of all respondents, 90.5% chose to send serum MOG-IgG antibody after the first event (59.7% serum, 36.4% CSF + serum). For acute treatment, 84.1% chose to give a 3-5-day course of high-dose IV steroids. Approaches to steroid tapering varied, with 33.0% choosing a 2-4-week taper, 27.2% choosing a 7-12-week taper, and 21% not offering a steroid taper. 56.6% of non-NIs chose to initiate maintenance therapy after the first episode while only 18.9% of NIs chose to do so. After the second episode, 98.3% of all respondents recommended starting maintenance therapy, with rituximab (RTX) (37.1%) being the most frequently chosen agent, followed by monthly IV immunoglobulin (IVIG) (25.6%) and azathioprine (17.1%). NIs selected monthly IVIG (50%) over RTX (27.3%). The duration of treatment in relapsing cases varied: 42.9% elected to maintain treatment for 2 years or less and 35.3% for more than 2 years, and 21.8% chose to continue treatment indefinitely.

Discussion: The survey demonstrated substantial variability in management decisions related to MOGAD among neurologists, reflecting current gaps in knowledge about therapies for MOGAD. Future efforts are needed to improve the uptake of knowledge and ensure that current guidelines are effectively translated into clinical practice.

探讨小儿MOG抗体相关疾病的治疗方法:一项神经科医师调查。
背景和目的:抗髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGADs)的诊断标准于2023年公布,但关于儿童MOGAD的最佳治疗策略存在争议。本研究的目的是评估神经科医生对小儿MOGAD的治疗方法和首选诊断调查。方法:于2024年4 - 10月通过《神经病学®临床实践》的Practice Current Section发布了一份针对儿科MOGAD治疗的调查问卷。通过美国神经病学学会(AAN)社交媒体平台、AAN网站和《神经病学®》印刷版的广告,以及在专业神经病学会议上共享的二维码,征集神经学家的反馈。问卷包括12个问题,评估儿童MOG-IgG抗体检测呈阳性后第一次和第二次神经炎症发作后的临床决策。人口统计问题也包括在内。使用描述性统计对反馈进行评估。对自我认定为神经免疫学家(NIs)者和非神经免疫学家进行了比较分析。结果:共346名神经科医师完成调查,其中全科医师占52.3%,专科医师占32.1%,其他神经科15.6%。在所有应答者中,90.5%的人选择在首次发病后送血清MOG-IgG抗体(59.7%血清,36.4% CSF +血清)。对于急性治疗,84.1%的患者选择3-5天疗程的高剂量静脉注射类固醇。类固醇逐渐减少的方法各不相同,33.0%的人选择2-4周逐渐减少,27.2%的人选择7-12周逐渐减少,21%的人不提供类固醇逐渐减少。56.6%的非NIs患者在首次发作后选择开始维持治疗,而只有18.9%的NIs患者选择这样做。第二次发作后,98.3%的受访者建议开始维持治疗,其中利妥昔单抗(RTX)(37.1%)是最常选择的药物,其次是每月静脉注射免疫球蛋白(IVIG)(25.6%)和硫唑嘌呤(17.1%)。NIs选择每月IVIG(50%)多于RTX(27.3%)。复发病例的治疗时间各不相同:42.9%选择维持治疗2年及以下,35.3%选择维持治疗2年以上,21.8%选择无限期继续治疗。讨论:调查显示神经科医生在与MOGAD相关的管理决策方面存在很大差异,反映了目前关于MOGAD治疗的知识差距。未来需要努力提高知识的吸收,并确保现行指南有效地转化为临床实践。
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来源期刊
Neurology. Clinical practice
Neurology. Clinical practice CLINICAL NEUROLOGY-
CiteScore
4.00
自引率
0.00%
发文量
77
期刊介绍: Neurology® Genetics is an online open access journal publishing peer-reviewed reports in the field of neurogenetics. The journal publishes original articles in all areas of neurogenetics including rare and common genetic variations, genotype-phenotype correlations, outlier phenotypes as a result of mutations in known disease genes, and genetic variations with a putative link to diseases. Articles include studies reporting on genetic disease risk, pharmacogenomics, and results of gene-based clinical trials (viral, ASO, etc.). Genetically engineered model systems are not a primary focus of Neurology® Genetics, but studies using model systems for treatment trials, including well-powered studies reporting negative results, are welcome.
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