Department of Pediatrics, Section of Neurology and Developmental Neuroscience, Baylor College of Medicine at Texas Children's Hospital, Houston, Texas.
Department of Pediatrics, Section of Neurology and Developmental Neuroscience, Baylor College of Medicine at Texas Children's Hospital, Houston, Texas.
Pediatr Neurol. 2023 Oct;147:36-43. doi: 10.1016/j.pediatrneurol.2023.07.002. Epub 2023 Jul 11.
We describe a cohort of children with severe myelin oligodendrocyte glycoprotein (MOG)-IgG-associated cerebral cortical encephalitis (CCE), manifesting with bilateral cortical cytotoxic edema and critical neurological illness.
We retrospectively reviewed our pediatric MOG antibody-associated disease (MOGAD) database and identified patients with specific radiographic pattern of bilateral, multifocal cortical cytotoxic lesions. We collected demographic, clinical, and outcomes data from these patients and compared select variables with radiographically distinct cerebral MOGAD syndromes (case-control analysis). We assessed the correlation of quantitative clinical variables with severity/outcomes measures using simple linear regression.
Sixty-five of 88 total MOGAD cases had cerebral disease, and six of 88 met inclusion criteria for fulminant CCE (f-CCE). Age range was 2 to 7 years; five of six were male. Six of six were critically ill with severe encephalopathy and seizures, two of six required barbiturate coma, and two of six required invasive intracranial pressure monitoring. Six of six required treatment escalation beyond steroids. Four of six had favorable outcome; two of six had moderate-severe disability. Compared with other cerebral MOGAD cases (n = 59), children with f-CCE were more likely to have critical illness and poor neurological outcomes scores. Neurofilament light chain and treatment latency positively correlated with intensive care unit length of stay and outcomes scores; cerebrospinal fluid (CSF) white blood cell count and neutrophil-to-lymphocyte ratio did not.
Pediatric CCE with bilateral cytotoxicity is associated with more fulminant disease and worse outcomes than other cerebral MOGAD syndromes.
我们描述了一组严重髓鞘少突胶质细胞糖蛋白（MOG）-IgG 相关大脑皮质脑炎（CCE）患儿，表现为双侧皮质细胞毒性水肿和严重神经疾病。
我们回顾性地查阅了我们的儿科 MOG 抗体相关疾病（MOGAD）数据库，并确定了具有双侧、多灶性皮质细胞毒性病变特定放射影像学模式的患者。我们从这些患者中收集了人口统计学、临床和结局数据，并与具有不同放射影像学特征的大脑 MOGAD 综合征患者进行了选择变量的比较（病例对照分析）。我们使用简单线性回归评估了定量临床变量与严重程度/结局测量之间的相关性。
在总共 88 例 MOGAD 病例中，有 65 例出现脑部疾病，88 例中有 6 例符合暴发性 CCE（f-CCE）的纳入标准。年龄范围为 2 至 7 岁；6 例均为男性。6 例均为危重症，伴有严重脑病和癫痫发作，2 例需要巴比妥昏迷，2 例需要有创颅内压监测。6 例均需要除类固醇以外的治疗升级。4 例结局良好；2 例有中重度残疾。与其他大脑 MOGAD 病例（n=59）相比，f-CCE 患儿更有可能患有严重疾病和较差的神经结局评分。神经丝轻链和治疗潜伏期与重症监护病房住院时间和结局评分呈正相关；脑脊液（CSF）白细胞计数和中性粒细胞与淋巴细胞比值则无相关性。
与其他大脑 MOGAD 综合征相比，双侧细胞毒性的小儿 CCE 与更严重的疾病和更差的结局相关。
