抗富亮氨酸胶质瘤失活1蛋白抗体脑炎12例临床特点  

Clinical characteristics of 12 patients with anti-leucine rich glioma inactivated protein 1 encephalitis

作　　者：孙桂芳[1] 胡文涛[1] 袁志浩[2] 李京红[3] 刘希[1] 王梦涵[1] 张博爱[1] 卢宏[1] 

机构地区：[1]郑州大学第一附属医院神经内科,450052 [2]郑州大学第一附属医院急诊外科,450052 [3]郑州大学第一附属医院神经内科实验室,450052

出　　处：《中华神经科杂志》2018年第2期91-96,共6页Chinese Journal of Neurology

基　　金：国家自然科学基金项目资助项目（81501111）;河南省教育厅高校重点科研项目（18A320065）

摘　　要：目的 总结抗富亮氨酸胶质瘤失活1蛋白（LGI1）抗体脑炎患者的临床表现，并随访观察其预后。方法 收集2015年6月至2016年12月郑州大学第一附属医院收治的12例抗LGI1受体脑炎患者，总结其临床表现和脑电图、实验室检查、影像学检查结果，进行随访，并观察预后。采用改良Rankin量表（mRS）评分进行治疗前后评估。结果 12例患者中表现为近记忆力下降者10例，定向力下降7例，全身强直阵挛性发作9例，面臂肌张力障碍发作7例，低钠血症5例，精神行为异常1例，易惊醒1例，睡眠增多3例，语言障碍4例，饮水呛咳、吞咽困难2例，头痛1例，头昏沉感2例，乏力2例，共济失调2例，合并心动过缓3例，小便障碍2例，肠梗阻1例，腹泻1例。入院mRS评分3分者8例，4分者4例。脑电图异常者6例，多为局灶性或弥漫性慢波，可伴异常癫痫波发放。头颅MRI异常者4例，主要累及海马、颞叶内侧、基底节区，1例患者因植入心脏起搏器不能做MRI检查。2例患者合并肺部结节，1例合并甲状腺抗体阳性和类风湿因子增高。治疗后6～24个月随访结果显示，无1例死亡，mRS评分2分者2例，1分9例，0分1例，遗留症状为记忆力障碍、睡眠增多、面臂肌张力障碍发作。结论 抗LGI1脑炎是可治性疾病，主要临床表现为癫痫发作、高级智能下降、低钠血症等，免疫治疗可明显改善症状，预后相对较好。Objective To analyze the clinical features and prognosis of anti-leucine rich glioma inactivated protein 1 （LGI1） encephalitis.Methods Twelve encephalitis patients with anti-LGI1 antibodies were collected from the First Affiliated Hospital of Zhengzhou University from June 2015 to December 2016. The clinical manifestations, electroencephalogram, laboratory examination and imaging findings were summarized and the prognosis was observed. The modified Rankin Scale （mRS） was used for evaluation before and after treatment.Results The major clinical features included memory deficit （10/12）, spatial disorientation （7/12）, epilepsy with generalized tonic-clonic seizures （9/12）, faciobrachial dystonic seizures （7/12）, hyponatremia （5/12）, mental and behavioral abnormalites （1/12）, light sleep （1/12）, increased sleep （3/12）, aphasis （4/12）, dysphagia, choking （2/12）, headache（1/12）, dizziness （2/12）, fatigue （2/12）, ataxia（2/12）, bradycardia （3/12）, urinary disorders （2/12）, intestinal obstruction （1/12）, diarrhea （1/12）. Admission mRS score was found to be three in eight cases, four in four cases. The abnormal electroencephalogram was found in six cases, mainly manifested as focal or diffuse slow wave, some accompanied by epileptic wave. MRI scan of brain showed abnormal signals in four cases, mainly involved medial temporal lobe, hippocampus, basal ganglia, while one patient avoided MRI scan due to implantation of pacemaker. Two patients presented with pulmonary nodules, one case with positive thyroid antibody and increased rheumatoid factor. The follow-up after treatment showed no one died; mRS score was two in two cases, one in nine cases and zero in one case; the sequelae were memory deficit, increased sleep, faciobrachial dystonic seizures.Conclusions Anti-LGI1 encephalitis is a treatable disease, cardinal clinical features of which are seizures, cognitive disorders, hyponatremia. Immunotherapy can improve the symptoms of the disease sign

关 键 词：神经系统自身免疫疾病 脑炎 癫痫 强直阵挛性 记忆障碍 低钠血症 

分 类 号：R742.9[医药卫生—神经病学与精神病学]