Fellmeth Ruth Helena, Kousoulos Lampros, Korenke George Christoph, Christen Hans-Jürgen, Monazahian Masyar, Dargvainiene Justine, Wandinger Klaus-Peter, Leypoldt Frank, Rostásy Kevin
Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany.
Department of Neuropediatrics, University Children's Hospital, Klinikum Oldenburg, Oldenburg, Germany.
Neuropediatrics. 2025 Aug;56(4):226-233. doi: 10.1055/a-2579-6247. Epub 2025 Apr 10.
Encephalitis in children is a serious inflammatory brain disease caused by infectious or autoimmune-mediated processes. The frequency of autoimmune variants in pediatric populations is not entirely clear.To study the frequency of myelin oligodendrocyte glycoprotein (MOG) antibody (ab)-mediated autoimmune encephalitis (AE) in children included in the Meningitis/Encephalitis register of Lower Saxony (MERIN).Medical records of 1,341 children treated between 2011 and 2020 in two large children's hospitals participating in a prospective study on encephalitis (MERIN) were reviewed. Children meeting diagnostic criteria for possible AE were finally included if serum samples and informed re-consent were available. Children with pathogen-mediated encephalitis were also included as controls. All available serum samples were tested for MOG- and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies (abs) using cell- and tissue-based assay (TBA).We included 55 children of whom 16 had pathogen-associated meningoencephalitis. Thirty-nine out of fifty-five children were classified as possible AE and 3/39 fulfilled the criteria for MOG ab-associated disease (MOGAD). No patients' sera harbored NMDAR abs. However, 5/39 patients fulfilled the criteria for probable, auto-ab-negative AE.In line with recent research our study suggests that ab-mediated AE and probable ab-negative AE are rare in children. The existing criteria seem suitable for identifying patients with AE but should include the testing of serum MOG abs. Further yet unknown abs may play a role in children with AE.
儿童脑炎是一种由感染或自身免疫介导的过程引起的严重炎症性脑病。儿科人群中自身免疫性变体的发生率尚不完全清楚。为了研究下萨克森州脑膜炎/脑炎登记册(MERIN)中儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体(ab)介导的自身免疫性脑炎(AE)的发生率。回顾了2011年至2020年期间在两家大型儿童医院接受治疗的1341名儿童的病历,这些医院参与了一项关于脑炎的前瞻性研究(MERIN)。如果有血清样本并获得知情再同意,最终纳入符合可能AE诊断标准的儿童。病原体介导的脑炎患儿也作为对照纳入。使用基于细胞和组织的检测方法(TBA)对所有可用血清样本进行MOG和抗N-甲基-D-天冬氨酸受体(NMDAR)抗体(abs)检测。我们纳入了55名儿童,其中16名患有病原体相关的脑膜脑炎。55名儿童中有39名被归类为可能的AE,3/39名符合MOG ab相关疾病(MOGAD)的标准。没有患者血清中含有NMDAR abs。然而,5/39名患者符合可能的自身抗体阴性AE的标准。与最近的研究一致,我们的研究表明,抗体介导的AE和可能的抗体阴性AE在儿童中很少见。现有的标准似乎适合识别AE患者,但应包括血清MOG abs检测。其他尚不清楚的抗体可能在AE儿童中起作用。