儿童 MOG-Ab 相关性脑炎：支持早期识别和治疗。
Pediatric MOG-Ab-Associated Encephalitis: Supporting Early Recognition and Treatment.
机构信息
From the Department of Neurology (N.N.K., O.A.-M., Y.H.), Great Ormond Street Hospital for Children NHS Foundation Trust; Department of Neuroinflammation (N.N.K., D.C., O.A.-M., C.H., O.C., Y.H.), Institute of Neurology, University College London; Children's Neurosciences (M.E., V.L., M.L., T.R.), Evelina London Children's Hospital, Guy's and St Thomas NHS Foundation Trust; Department of Women and Children's Health (M.E., M.L., T.R.), School of Life Course Sciences (SoLCS), King's College London; Department of Paediatrics (A.S., S.R., J.P.), Children's Hospital, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust; Department of Paediatric Neurology (M.V.C.), Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust; Translational and Clinical Research Sir James Spence Institute (R.F.), University of Newcastle, Royal Victoria Infirmary; Department of Neurology (R.F.), Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust; Department of Neurology (R.K.), Alder Hey Children's Hospital, Alder Hey Children's NHS Foundation Trust, Liverpool; Department of Paediatric Neurology (D.R., Siobhan West), Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust; Department of Neurology (E.W., Sukhvir Wright), Birmingham Children's Hospital, Birmingham Women's and Children's NHS Foundation Trust; Department of Neuroradiology (A.B., K.M.), Great Ormond Street Hospital, Great Ormond Street Hospital Trust, London, United Kingdom; Department of Neurology (E.P.F.), Laboratory Medicine and Pathology and Center for Multiple Sclerosis and Autoimmune Neurology, Rochester, MN; NIHR University College London Hospitals Biomedical Research Centre (O.C.); and Department of Neuroinflammation (O.C.), National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, United Kingdom.
出版信息
Neurol Neuroimmunol Neuroinflamm. 2024 Dec;11(6):e200323. doi: 10.1212/NXI.0000000000200323. Epub 2024 Oct 11.
BACKGROUND AND OBJECTIVES
Antibodies to myelin oligodendrocyte glycoprotein (MOG-Ab) have recently been reported in patients with encephalitis who do not fulfill criteria for acute disseminated encephalomyelitis (ADEM). We evaluated a cohort of these children and compared them with children with ADEM.
METHODS
This retrospective, multicenter cohort study comprised consecutive patients <18 years of age with MOG-Ab who fulfilled criteria for autoimmune encephalitis. These patients were stratified into (1) children not fulfilling criteria for ADEM (encephalitis phenotype) and (2) children with ADEM. Clinical/paraclinical data were extracted from the electronic records. Comparisons were made using the Mann-Whitney test and χ Fisher exact test for statistical analysis.
RESULTS
From 235 patients with positive MOG-Ab, we identified 33 (14%) with encephalitis and 74 (31%) with ADEM. The most common presenting symptoms in children with encephalitis were headache (88%), seizures (73%), and fever (67%). Infective meningoencephalitis was the initial diagnosis in 67%. CSF pleocytosis was seen in 79%. Initial MRI brain was normal in 8/33 (24%) patients. When abnormal, multifocal cortical changes were seen in 66% and unilateral cortical changes in 18%. Restricted diffusion was demonstrated in 43%. Intra-attack new lesions were seen in 7/13 (54%). When comparing with children with ADEM, children with encephalitis were older (median 8.9 vs 5.7 years, = 0.005), were more likely to be admitted to intensive care (14/34 vs 4/74, < 0.0001), were given steroid later (median 16.6 vs 9.6 days, = 0.04), and were more likely to be diagnosed with epilepsy at last follow-up (6/33 vs 1/74, = 0.003).
DISCUSSION
MOG-Ab should be tested in all patients with suspected encephalitis even in the context of initially normal brain MRI. Although exclusion of infections should be part of the diagnostic process of any child with encephalitis, in immunocompetent children, when herpes simplex virus CSF PCR and gram stains are negative, these features do not preclude the diagnosis of immune mediated disease and should not delay initiation of first-line immunosuppression (steroids, IVIG, plasma exchange), even while awaiting the antibody results.
背景与目的
髓鞘少突胶质细胞糖蛋白（MOG-Ab）抗体最近在不符合急性播散性脑脊髓炎（ADEM）标准的脑炎患者中被报道。我们评估了一组此类患儿，并与 ADEM 患儿进行了比较。
方法
本回顾性多中心队列研究纳入了符合自身免疫性脑炎标准的连续 MOG-Ab 阳性＜18 岁患儿。这些患儿分为（1）不符合 ADEM 标准的患儿（脑炎表型）和（2）ADEM 患儿。从电子病历中提取临床/辅助检查数据。采用 Mann-Whitney U 检验和χ² Fisher 确切概率法进行统计学分析。
结果
在 235 例 MOG-Ab 阳性患者中，我们发现 33 例（14%）为脑炎，74 例（31%）为 ADEM。脑炎患儿最常见的首发症状为头痛（88%）、癫痫发作（73%）和发热（67%）。67%的患儿最初诊断为感染性脑膜脑炎。79%的患儿有 CSF 细胞增多。33 例患儿中，8 例（24%）初始脑 MRI 正常。异常时，66%表现为多灶性皮质改变，18%为单侧皮质改变。43%的患儿出现弥散受限。13 例患儿中有 7 例（54%）在发作期出现新病灶。与 ADEM 患儿相比，脑炎患儿年龄较大（中位数 8.9 岁 vs 5.7 岁， = 0.005），更有可能入住重症监护病房（34 例中有 14 例 vs 74 例中有 4 例， < 0.0001），更晚接受类固醇治疗（中位数 16.6 天 vs 9.6 天， = 0.04），且在最后一次随访时更有可能被诊断为癫痫（33 例中有 6 例 vs 74 例中有 1 例， = 0.003）。
讨论
即使在初始脑 MRI 正常的情况下，也应在疑似脑炎的所有患者中检测 MOG-Ab。虽然在任何脑炎患儿的诊断过程中都应排除感染，但在免疫功能正常的患儿中，当单纯疱疹病毒 CSF PCR 和革兰染色为阴性时，这些特征并不能排除免疫介导疾病的诊断，不应因等待抗体结果而延迟一线免疫抑制（类固醇、IVIG、血浆置换）的启动，即使在等待抗体结果的同时。
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