Zhou J, Ding C H, Zhang W H, Zhuo X W, Li J W, Gong S, Guan H Z, Fang F, Zhu X Y, Cheng H, Ren X T
Department of Neurology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Yi Xue Za Zhi. 2020 Jul 7;100(25):1952-1955. doi: 10.3760/cma.j.cn112137-20200525-01644.
To describe the clinical features of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-positive cortical encephalitis in children. Patients who were hospitalized in Beijing Children's Hospital from June 2018 to October 2019, with positive MOG antibodies and phenotype of cortical encephalitis were retrospectively analyzed. Cell-based assays (CBAs) were used to test MOG antibodies. Five patients had the phenotype of cortical encephalitis during follow-up, with 3 females and 2 males. The age of onset ranged from 8 years to 12 years and 1 month. At the last follow-up, 3 cases exhibited a monophasic course and 2 cases were with relapse and remission courses. Six out of 8 episodes which had the phenotype of cortical encephalitis presented with seizures, among which 3 episodes had status epilepticus. None had recurrent seizures during remission. Other symptoms included fever (7/8), headache and vomiting (4/8), somnolence (3/8) and hemiplegia (1/8). Unilateral cortical swelling was observed in cerebral magnetic resonance imaging (MRI) of all patients, without any hemorrhage and necrosis. White blood cell (WBC) counts of cerebrospinal fluid increased, ranging from8×10(6)/L to 186×10(6)/L. All patients recovered well after treatment with intravenous immunogloblin and glucocorticoid. Two patients had relapses during follow-up and were additionally treated with mycophenolate mofetil. Anti-MOG antibodies can induce cortical encephalitis. In clinical setting, fever, headache and seizures are common, however, severe consciousness disturbance and local neurological deficits are rare in these patients. Cerebral MRI shows unilateral cortical swelling without any hemorrhage and necrosis. Usually, immunotherapy works well. No patients exist repeated seizures in remission, but some patients may have relapses.
描述儿童抗髓鞘少突胶质细胞糖蛋白（MOG）抗体阳性皮质脑炎的临床特征。回顾性分析2018年6月至2019年10月在北京儿童医院住院的、MOG抗体阳性且具有皮质脑炎表型的患者。采用细胞法检测MOG抗体。5例患者在随访期间出现皮质脑炎表型，其中女性3例，男性2例。发病年龄为8岁至12岁1个月。末次随访时，3例呈单相病程，2例呈复发缓解病程。8次出现皮质脑炎表型的发作中有6次表现为癫痫发作，其中3次为癫痫持续状态。缓解期均无癫痫复发。其他症状包括发热（7/8）、头痛和呕吐（4/8）、嗜睡（3/8）和偏瘫（1/8）。所有患者的脑磁共振成像（MRI）均显示单侧皮质肿胀，无任何出血和坏死。脑脊液白细胞（WBC）计数升高，范围为8×10⁶/L至186×10⁶/L。所有患者经静脉注射免疫球蛋白和糖皮质激素治疗后恢复良好。2例患者在随访期间复发，加用霉酚酸酯治疗。抗MOG抗体可诱发皮质脑炎。在临床情况下，发热、头痛和癫痫发作常见，但这些患者严重意识障碍和局部神经功能缺损少见。脑MRI显示单侧皮质肿胀，无任何出血和坏死。通常，免疫治疗效果良好。缓解期无患者癫痫复发，但部分患者可能复发。
