Tang Xiaolu, Zhang Weihua, Zhou Anna, Ren Changhong, Ren Xiaotun, Yin Guangheng, Lv Yanqiu, Cheng Hua, Peng Yun
Department of Radiology, Ministry of Education (MOE) Key Laboratory of Major Diseases in Children, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, No. 56 Nanlishi Road, Xicheng District, Beijing, 100045, China.
Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Pediatr Radiol. 2025 Jun 4. doi: 10.1007/s00247-025-06269-4.
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a neuroinflammatory disorder with a heterogeneous phenotype. MOGAD, presenting with meningoencephalitis, has been described, with limited data on the clinical and radiographic features in children.
We aimed to comprehensively investigate the clinical and radiographic characteristics of the MOG antibody-associated meningoencephalitis in children and to identify any magnetic resonance imaging (MRI) markers associated with relapse.
This was a single-center, retrospective study. All data for pediatric patients (age of onset < 18 years) diagnosed with MOGAD at our hospital from January 2018 to January 2024 were reviewed. Patients with initial clinicoradiological constellations consistent with meningoencephalitis were included in the analysis and subsequently classified into monophasic and multiphasic groups based on the presence or absence of clinical relapses.
A total of 53 children (23% of our MOGAD cohort; age range, 2.7-16.4 years; median age, 11.4 years; 21 females) were included; among them, 21 (40%) presented with a multiphasic course. Within the entire cohort, the common clinical manifestations included seizures (37 (70%)), headache (35 (66%)), and fever (31 (58%)). MRI revealed unilateral involvement in 35 (66%) and bilateral involvement in 18 (34%). Frontal lobe involvement (45 (85%)) was the most predominant MRI pattern. On T2-weighted fluid-attenuated inversion recovery (FLAIR) images, cortical hyperintensity was observed in 46 (87%) patients, leptomeningeal linear hyperintensity in 28 (53%), and subcortical FLAIR hypointensity in 51 (96%). Diffusion restriction (4 (8%)) was uncommon. Meningeal enhancement was observed in 88% (38/43). Compared with the monophasic group, the multiphasic group demonstrated greater proportions of parietal (P = 0.023), occipital lobe (P = 0.016), and hemispheric (P = 0.023) involvement, as well as a greater median number of involved lobes (P = 0.011). Multivariate logistic regression analysis, adjusted for age and sex, revealed that a greater number of involved lobes was an independent risk factor for relapse (P = 0.010, odds ratio (OR) = 1.547).
Subcortical FLAIR hypointensity, also known as the "dark white matter" sign, is a particularly prominent imaging finding in children with MOG antibody-associated meningoencephalitis. In pediatric patients with epilepsy of unknown etiology, the concurrent presence of meningocortical abnormalities and the "dark white matter" sign on MRI is highly suggestive of this phenotype. A more extensive meningocortical lesion burden is associated with a higher risk of relapse.
髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）是一种具有异质性表型的神经炎症性疾病。已有关于表现为脑膜脑炎的MOGAD的描述，但儿童患者临床及影像学特征的数据有限。
我们旨在全面研究儿童MOG抗体相关脑膜脑炎的临床和影像学特征，并确定与复发相关的磁共振成像（MRI）标志物。
这是一项单中心回顾性研究。回顾了2018年1月至2024年1月在我院诊断为MOGAD的儿科患者（发病年龄<18岁）的所有数据。将初始临床和放射学表现符合脑膜脑炎的患者纳入分析，并根据是否存在临床复发分为单相和多相组。
共纳入53例儿童（占我们MOGAD队列的23%；年龄范围2.7 - 16.4岁；中位年龄11.4岁；21例女性）；其中21例（40%）表现为多相病程。在整个队列中，常见临床表现包括癫痫发作（37例（70%））、头痛（35例（66%））和发热（31例（58%））。MRI显示单侧受累35例（66%），双侧受累18例（34%）。额叶受累（45例（85%））是最主要的MRI表现形式。在T2加权液体衰减反转恢复（FLAIR）图像上，46例（87%）患者观察到皮质高信号，28例（53%）患者观察到软脑膜线状高信号，51例（96%）患者观察到皮质下FLAIR低信号。扩散受限（4例（8%））不常见。88%（38/43）的患者观察到脑膜强化。与单相组相比，多相组顶叶（P = 0.023）、枕叶（P = 0.016）和半球（P = 0.023）受累比例更高，且受累叶的中位数更多（P = 0.011）。在对年龄和性别进行校正的多因素逻辑回归分析中，受累叶数量更多是复发的独立危险因素（P = 0.010，比值比（OR）= 1.547）。
皮质下FLAIR低信号，即所谓的“暗白质”征，是儿童MOG抗体相关脑膜脑炎中特别突出的影像学表现。在病因不明的癫痫儿科患者中，MRI上脑膜皮质异常与“暗白质”征同时出现高度提示该表型。更广泛的脑膜皮质病变负荷与更高的复发风险相关。
