富亮氨酸胶质瘤失活1蛋白抗体脑炎的临床特点  

Clinical characteristics of leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis

作　　者：贺菲菲[1] 叶静[1] 徐文灯 

机构地区：[1]首都医科大学宣武医院神经内科,北京100050

出　　处：《中华神经科杂志》2016年第1期26-29,共4页Chinese Journal of Neurology

基　　金：国家科技支撑计划项目（2013BAI07B01）

摘　　要：目的分析富亮氨酸胶质瘤失活1蛋白（LGIl）抗体脑炎的临床和影像学表现。方法收集2012年1月至2015年1月首都医科大学宣武医院确诊的LGIl抗体脑炎14例，分析其临床资料、头颅MRI、腰椎穿刺和生化检查结果，总结其临床特点。结果14例LGIl抗体脑炎患者平均年龄（48．93±15．60）岁（27～67岁），男女比为2．5：1，所有患者均出现短期内近记忆力下降，9例出现痴呆，12例有癫痫发作，8例有面臂肌张力障碍发作（FBDS），10例有精神症状，8例有睡眠障碍。2例患者于疾病严重时转入ICU，并行机械辅助通气。13例头颅MRI异常，主要累及颞叶、海马。6例腰椎穿刺结果异常，8例出现低钠血症（血钠〈135mmol／L），5例合并其他血清自身抗体阳性。所有患者行肿瘤筛查均未发现肿瘤。随访2年，3例复发。结论LGll抗体脑炎是一种以近记忆力减退、FBDS及低钠血症为主要临床特征的自身免疫性脑炎，头颅MRI以颞叶、海马受累为主，很少合并肿瘤，可复发。Objective To analyze the clinical characteristics of leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis. Methods Fourteen patients diagnosed as leucine-rich glioma- inactivated protein 1 antibody-associated encephalitis in Capital Medical University Xuanwu Hospital from January 2012 to January 2015 were recruited. The clinical manifestation, brain magnetic resonance imaging, cerebrospinal fluid findings and biochemical examination of these patients were analyzed. Results The case series of 14 patients had an average age of （48.93 ± 15.60） years （range 27 -67 years） with a male to female ratio of 2. 5： 1. All patients presented with short-term memory loss. Nine patients experienced dementia. Additionally, among the 14 patients, 12 experienced seizures, 8 experienced faciobrachial dystonic seizures, 10 had psychiatric symptoms, and 8 showed sleep dysfunction. Two patients were transferred into intensive care unit because of deteriorating symptoms and were provided operated mechanical ventilation. Thirteen of 14 patients exhibited abnormalities in their brain magnetic resonance imaging, with lesions in temporal lobe and hippocampus. Six patients had abnormal cerebrospinal fluid findings, 8 patients showed hyponatremia （serum Na^＋ 〈 135 mmol/L） , while 5 patients co-existed with other autoantibodies. Screening for malignant tumors revealed normal findings. During 2 years follow-up, 3 patients relapsed. Conclusions Leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis is an autoimmune encephalitis characterized by short-term memory loss, faciobrachial dystonic seizures and hyponatremia. Lesions in brain MRI always involve in temporal lobe and hippocampus. This disease can relapse and is seldom associated with tumor.

关 键 词：神经系统自身免疫疾病 脑炎 蛋白质类 磁共振成像 复发 

分 类 号：R742.9[医药卫生—神经病学与精神病学]