Myelin oligodendrocyte glycoprotein antibody-associated meningoencephalitis without cerebral parenchyma involvement on MRI: A single-centre paediatric cohort study.

IF 3 3区 医学 Q1 PEDIATRICS
Ren Changhong, Liu Ming, Zhou Anna, Zhou Ji, Zhuo Xiuwei, Tian Xiaojuan, Yang Xinying, Gong Shuai, Fang Fang, Ren Xiaotun, Zhang Weihua
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Abstract

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.

髓鞘少突胶质细胞糖蛋白抗体相关的脑膜脑炎,MRI无脑实质受累:一项单中心儿科队列研究。
由于脑膜脑炎与突出的脑膜累及最初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,零星病例已被报道。然而,大多数报告是病例报告和小样本队列。我们对2017年至2024年北京儿童医院无脑实质累及MOG抗体相关脑膜脑炎患儿进行回顾性分析。在回顾本中心320例MOG抗体阳性儿科患者的记录后,我们确定了18例(6%)患者(中位年龄7.2岁;女性,10岁)发病。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%)和ESR和CRP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例患者出现颅磁共振阴性,14例患者出现脑膜增强。14例患者抗生素治疗无改善,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。最后一次随访时,所有患者预后良好(mRS
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来源期刊
CiteScore
5.90
自引率
2.80%
发文量
367
审稿时长
3-6 weeks
期刊介绍: The European Journal of Pediatrics (EJPE) is a leading peer-reviewed medical journal which covers the entire field of pediatrics. The editors encourage authors to submit original articles, reviews, short communications, and correspondence on all relevant themes and topics. EJPE is particularly committed to the publication of articles on important new clinical research that will have an immediate impact on clinical pediatric practice. The editorial office very much welcomes ideas for publications, whether individual articles or article series, that fit this goal and is always willing to address inquiries from authors regarding potential submissions. Invited review articles on clinical pediatrics that provide comprehensive coverage of a subject of importance are also regularly commissioned. The short publication time reflects both the commitment of the editors and publishers and their passion for new developments in the field of pediatrics. EJPE is active on social media (@EurJPediatrics) and we invite you to participate. EJPE is the official journal of the European Academy of Paediatrics (EAP) and publishes guidelines and statements in cooperation with the EAP.
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