首页|以皮质脑炎为主要表现的MOG抗体相关病4例并文献复习

以皮质脑炎为主要表现的MOG抗体相关病4例并文献复习

扫码查看
目的 通过报道以皮质脑炎为主要表现的抗髓鞘少突胶质细胞糖蛋白(MOG)抗体相关病(简称MOG-AD)4例,分析其临床特征及相关辅助检查等,并结合文献复习,以期为临床上该病的正确诊疗提供参考依据。方法 回顾性分析济宁医学院附属医院神经内科2020年10月至2023年2月收治的4例以皮质脑炎为主要表现的MOG-AD患者的临床表现、脑脊液细胞学、影像学、治疗和预后等特征。抗MOG抗体检测方法为基于细胞转染的间接免疫荧光法(CBA)。结果 本文中4例患者发病年龄为16~32岁,均以皮质脑炎为临床表现,其中3例患者以癫痫发作起病,1例患者于头痛发热15 d后出现首次癫痫发作。头部MRI上表现为皮质区脑组织增厚并多发脑沟裂池内异常信号影,FLAIR序列上呈相对高信号。血清抗MOG抗体检测均表示阳性。患者经激素和(或)免疫球蛋白治疗均好转出院,其中2例患者随访期出现复发。结论 MOG-AD可以皮质脑炎为主要表型,表现为头痛、发热、癫痫发作,初期易被诊断为"感染性脑膜脑炎或脑膜炎",头部MRI常表现为单侧或双侧皮质肿胀;免疫治疗有效,部分患者可出现复发;对于抗感染治疗效果差的皮质脑炎患者,需警惕该病,行血清MOG-IgG检查示"阳性"可明确诊断。
Four cases of MOG antibody-associated disease with cortical encephalitis as the main manifestation and review of the literature
Objective We report four cases of myelin oligodendrocyte glycoprotein(MOG)antibody-associated dis-ease(MOG-AD)with cortical encephalitis as the main manifestation.We analyse the clinical features and relevant ancil-lary tests,and review the literature in order to provide a reference basis for the proper clinical management of this disease.Methods The clinical manifestations,cerebrospinal fluid cytology,imaging,treatment and prognosis of four patients with MOG-AD with cortical encephalitis as the main manifestation,admitted to the Department of Neurology of the Affili-ated Hospital of Jining Medical University from October 2020 to February 2023,were retrospectively analyzed.The anti-MOG antibodies were detected by a cell-based assay(CBA).Results The four patients in this paper were aged 16-32 years and all presented with cortical encephalitis.Three of them started with seizures and one presented with his first sei-zure 15 d after headache and fever.The cranial MRI showed thickening of brain tissue in the cortical area and multiple ab-normal signal shadows in the sulcal pools,with relatively high signal on FLAIR images.Serum anti-MOG antibody tests were positive.The patients were treated with hormones and/or immunoglobulin and discharged,with two patients experi-encing relapses.Conclusion MOG-AD can have a predominantly cortical encephalitis phenotype,presenting with head-ache,fever and seizures.The initial diagnosis is easily made as"infective meningoencephalitis or meningitis",and cra-nial MRI often shows unilateral or bilateral cortical swelling;immunotherapy is effective,but some patients may experi-ence recurrence;for patients with cortical encephalitis who are poorly treated with anti-infective therapy,the disease needs to be alerted,and a"positive"serum MOG-IgG test can clarify the diagnosis.

Myelin oligodendrocyte glycoproteinCortical encephalitisMOG antibody-related disordersepilepsy

史亚茹、褚旭、高明康、王金林、王琳

展开 >

济宁医学院临床医学院,山东 济宁 272002

济宁医学院附属医院,山东 济宁 272000

髓鞘少突胶质细胞糖蛋白 皮质脑炎 MOG抗体相关疾病 癫痫

济宁市重点研发计划项目山东省中医药科技项目

2022YXNS0462021M157

2024

中风与神经疾病杂志
吉林大学

中风与神经疾病杂志

CSTPCD
影响因子:0.754
ISSN:1003-2754
年,卷(期):2024.41(9)