抗LGI1抗体脑炎7例患者的临床、影像学及预后特点分析  

Clinical,imaging and prognostic characteristics of seven patients with anti-leucine-rich glioma-inactivated-1 antibody encephalitis

作　　者：安利 刘升阳 姚生 孙辰婧 刘建国 AN Li;LIU Shengyang;YAO Sheng;SUN Chenjing;LIU Jianguo(不详;Department of Anesthesiology,The Six Medical Center of PLA of Chinese Hospital,Beijing 100048,China)

机构地区：[1]中国人民解放军总医院第六医学中心神经内科,100048 [2]解放军医学院,100039 [3]中国人民解放军总医院第六医学中心麻醉科,100048

出　　处：《中国神经免疫学和神经病学杂志》2024年第3期174-179,共6页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的探讨抗富亮氨酸重复序列胶质瘤失活蛋白1(leucine-rich glioma-inactivated-1,LGI1)抗体脑炎患者的临床、实验室检查、影像学、治疗及预后特点。方法回顾性收集2020年6月至2023年8月就诊于解放军总医院第六医学中心确诊的7例抗LGI1抗体脑炎患者的临床资料,分析其临床、实验室检查、影像学、治疗及转归特点。结果(1)临床表现:癫痫发作、精神行为异常及睡眠障碍6例,面臂肌张力障碍样发作(faciobrachial dystonic seizure,FBDS)3例,Morvan综合征1例,癫痫发作伴肌无力综合征1例。(2)实验室检查:血和脑脊液LGI1抗体均阳性5例,血和脑脊液LGI1和Caspr2抗体均阳性1例,血LGI1、mGluR5、Titin抗体阳性并脑脊液LGI1抗体阳性1例。低钠血症7例;脑脊液压力、白细胞计数及蛋白均正常7例;脑电图检查轻度异常4例,主要表现为较多慢波,正常3例。(3)头颅MRI:单侧或双侧海马异常信号4例,右侧额叶梗死1例,双侧额颞枕皮层下异常信号1例,无异常1例。(4)治疗及预后:糖皮质激素、大剂量静脉注射免疫球蛋白、免疫抑制剂及抗癫痫治疗7例,症状均缓解。结论抗LGI1抗体脑炎主要表现为精神行为异常、顽固性癫痫、睡眠障碍及FBDS,可叠加Caspr2、mGluR5及Titin抗体阳性,低钠血症多见;影像学主要表现为单侧或双侧海马病变;免疫调节治疗效果好,确诊后应早期启动免疫治疗。Objective To investigate the clinical,laboratory,imaging,therapeutic and prognostic features of patients with anti-leucine-rich glioma-inactivated-1(LGI1)antibody encephalitis.Methods Clinical data of 7 patients with anti-LGI1 antibody encephalitis diagnosed in the Sixth Medical Center of the General Hospital of the People's Liberation Army from June 2020 to August 2023 were retrospectively collected.Their clinical,laboratory,imaging,treatment and outcome characteristics were analyzed.Results(1)Clinical manifestations:6 cases with seizures,psychobehavioral disorders and sleep disorders,3 cases with faciobrachial dystonic seizure(FBDS),1 case with Morvan syndrome,and 1 case with seizure and myasthenic syndrome.(2)Laboratory examination:5 cases were double positive for LGI1 antibodies in blood and cerebral spinal fluid(CSF),1 case was positive for LGI1 and Caspr2 antibodies in blood and CSF,and 1 case was positive for LGI1,mGluR5,Titin antibodies in blood,and LGI1 antibodies in CSF.Seven cases had hyponatremia.CSF pressure,white blood cell count and protein were normal in all 7 cases.Electroencephalogram examination showed mild abnormality in 4 cases,mainly manifested as more slow wave,and normal in 3 cases.(3)Head MRI:4 cases had unilateral or bilateral abnormal hippocampal signals,1 case had right frontal infarct,1 case had bilateral frontotemporal occipital abnormal signals,and 1 case was normal.(4)Treatment and prognosis:All 7 patients were treated with glucocorticoids,intravenous immunoglobulin,immunosuppressants and antiepileptic therapy,and their symptoms were relieved.Conclusions Anti-LGI1 antibody encephalitis is mainly characterized by mental and behavioral abnormalities,intractable epilepsy,sleep disorders and FBDS,which can be combined with positive Caspr2,mGluR5 and Titin antibodies.Hyponatremia is common.The main imaging findings were unilateral or bilateral hippocampal lesions.The effect of immunomodulatory therapy is good,and immunotherapy should be started early after diagnosis.

关 键 词：富亮氨酸重复序列胶质瘤失活蛋白1 抗LGI1抗体脑炎 Morvan综合征 肌无力综合征 自身免疫性脑炎 

分 类 号：R742.9[医药卫生—神经病学与精神病学]