Changhong Ren, Ming Liu, Anna Zhou, Ji Zhou, Xiuwei Zhuo, Xiaojuan Tian, Xinying Yang, Shuai Gong, Fang Fang, Xiaotun Ren, Weihua Zhang
Department of Neurology, National Center for Children'S Health, Beijing Children'S Hospital, Capital Medical University, No.56, Nan Li Shi Road, Beijing, 100045, China.
Eur J Pediatr. 2025 Apr 2;184(5):275. doi: 10.1007/s00431-025-06071-2.
Since meningoencephalitis with prominent meningeal involvement was first described as a rare phenotype of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), sporadic cases have been reported. However, most of the reports were case reports and small sample cohorts. We conducted a retrospective analysis of paediatric patients with MOG antibody-associated meningoencephalitis without cerebral parenchyma involvement on imaging at Beijing Children's Hospital between 2017 and 2024. Upon reviewing the records of 320 MOG antibody-positive paediatric patients in our centre, we identified 18 (6%) patients (median age, 7.2 years; female, 10) with disease onset. The common clinical symptoms were fever (94%), headache (56%), seizure (44%) and vomiting (44%). Most patients had leukocytosis (78%) and elevated ESR and CRP levels (61%). Cerebrospinal fluid analysis revealed intracranial hypertension (28%), leukocytosis (100%) and elevated protein levels (33%). Negative cranial magnetic resonance images were observed in 4 patients, and meningeal enhancement was observed in 14 patients. Fourteen patients showed no improvement with antibiotic therapy but responded effectively to immunotherapy. After a median follow-up of 22 months, 4 (22%) of the 18 patients experienced relapse. At the time of the last follow-up, all patients had a good prognosis (mRS < 2). Conclusion: Meningoencephalitis without parenchymal involvement on MRI represents an underrecognized MOGAD phenotype. The incorporation of this entity into diagnostic criteria and the initiation of early immunotherapy could increase diagnostic sensitivity and reduce relapse risk in children.
自从脑膜脑炎伴显著脑膜受累首次被描述为髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的一种罕见表型以来,已有散发病例报道。然而,大多数报告为病例报告和小样本队列研究。我们对2017年至2024年在北京儿童医院就诊的MOG抗体相关脑膜脑炎且影像学检查无脑实质受累的儿科患者进行了回顾性分析。在查阅本中心320例MOG抗体阳性儿科患者的记录时,我们确定了18例(6%)发病患者(中位年龄7.2岁;女性10例)。常见临床症状为发热(94%)、头痛(56%)、癫痫发作(44%)和呕吐(44%)。大多数患者有白细胞增多(78%)以及血沉和C反应蛋白水平升高(61%)。脑脊液分析显示颅内高压(28%)、白细胞增多(100%)和蛋白水平升高(33%)。4例患者头颅磁共振成像为阴性,14例患者有脑膜强化。14例患者抗生素治疗无效,但免疫治疗有效。中位随访22个月后,18例患者中有4例(22%)复发。在最后一次随访时,所有患者预后良好(改良Rankin量表评分<2)。结论:MRI无实质受累的脑膜脑炎是一种未被充分认识的MOGAD表型。将该实体纳入诊断标准并尽早开始免疫治疗可提高儿童诊断的敏感性并降低复发风险。