Yao Tianxin, Zeng Qianqian, Xie Yuanyuan, Bi Fangfang, Zhang Le, Xiao Bo, Zhou Jinxia
Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410002, China.
Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410002, China;; Hunan Key Laboratory of Aging Biology, Xiangya Hospital, Central South University, Changsha, 410002, China.
Mult Scler Relat Disord. 2022 Apr;60:103727. doi: 10.1016/j.msard.2022.103727. Epub 2022 Mar 5.
To describe the clinical and neuroimaging features, treatment response and outcomes of adult myelin oligodendrocyte glycoprotein (MOG) antibody-associated cortical encephalitis.
In this retrospective study, adult patients fulling the criteria of encephalitis but not fulfilling those of ADEM and tested positive for serum MOG antibody were recruited from Xiangya Hospital, Central South University (2019-2021). Clinical symptoms, laboratory data, imaging, and outcomes were analyzed.
Eleven MOG antibody-associated cortical encephalitis patients consisting of 4 female (36.4%) and 7 male (63.6%) were included with a median onset age of 27 years (ranging: 16-32 years). Fever (8/11), headache (9/11) and seizures (7/11) were the most common symptoms of adult MOG cortical encephalitis, and generalized seizure was the dominant seizure type (4/7). Increased intracranial pressure (5/10, median 280 mm H2O, ranging 240-380 mm H2O), CSF pleocytosis (5/10, median 48 cells/μL, ranging: 18-1800 cells/μL), and protein elevation (4/10, median 0.67 g/L, ranging: 0.46-1.92 g/L) were common. Serum MOG antibody was detected in all patients and the coexistence of CSF NMDAR antibody was found in one case. Imaging showed abnormal brain MRI in 10 patients (90.9%) and 10/10 had the cortical involvement. 8/10 had unilateral lesions while 2/10 had bilateral lesions. 2/10 had the juxta cortical white matter lesions and 2/10 had the corpus callosum lesions. No involvement of deep gray matter or other white matter structure was noted. 5/11 had leptomeningeal and/or lesional enhancements. 10/11 patients had favorable outcomes and 2/11 had clinical relapses with persisting MOG antibody positive during the median follow-up interval of 10 months (ranging: 3-23months).
Atypical clinical features for demyelinating diseases including seizures, remarkably increased intracranial pressure, pleocytosis, and protein elevation are common in MOG cortical encephalitis. Involvement of the corpus callosum and cortex around the midline could be unique imaging features of MOG antibody-associated bilateral cortical encephalitis.
描述成人髓鞘少突胶质细胞糖蛋白（MOG）抗体相关皮质脑炎的临床和神经影像学特征、治疗反应及预后。
在这项回顾性研究中，从中南大学湘雅医院招募了符合脑炎标准但不符合急性播散性脑脊髓炎（ADEM）标准且血清MOG抗体检测呈阳性的成年患者（2019 - 2021年）。对临床症状、实验室数据、影像学检查及预后进行分析。
纳入11例MOG抗体相关皮质脑炎患者，其中女性4例（36.4%），男性7例（63.6%），发病年龄中位数为27岁（范围：16 - 32岁）。发热（8/11）、头痛（9/11）和癫痫发作（7/11）是成人MOG皮质脑炎最常见的症状，全身性癫痫发作是主要的发作类型（4/7）。颅内压升高（5/10，中位数280 mm H₂O，范围240 - 380 mm H₂O）、脑脊液细胞增多（5/10，中位数48个细胞/μL，范围：18 - 1800个细胞/μL）和蛋白升高（4/10，中位数0.67 g/L，范围：0.46 - 1.92 g/L）较为常见。所有患者血清MOG抗体均呈阳性，1例患者脑脊液中同时存在N -甲基 - D -天冬氨酸受体（NMDAR）抗体。影像学检查显示10例患者（90.9%）脑MRI异常，10/10有皮质受累。8/10为单侧病变，2/10为双侧病变。2/10有皮质下白质病变，2/10有胼胝体病变。未发现深部灰质或其他白质结构受累。5/11有软脑膜和/或病灶强化。10/11患者预后良好，2/11在中位随访期10个月（范围：3 - 23个月）内出现临床复发，MOG抗体持续阳性。
MOG皮质脑炎常见脱髓鞘疾病的非典型临床特征，包括癫痫发作、颅内压显著升高、细胞增多和蛋白升高。胼胝体和中线周围皮质受累可能是MOG抗体相关双侧皮质脑炎的独特影像学特征。
