探讨儿童抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关脑炎的临床特征，并进行文献复习。

选择2016年3月至11月，于首都儿科研究所附属儿童医院及北京天坛医院神经内科住院的2例抗LGI1抗体相关脑炎患儿(患儿1、患儿2)为研究对象，进行病例资料分析并总结其临床特征。设定检索策略：以"富亮氨酸胶质瘤失活1蛋白""免疫性脑炎""LGI1""leucine-rich glioma-inactivated 1 protein"及"autoimmune diseases of the nervous system"为关键词，对万方数据知识服务平台、医知网、PubMed文献数据库、美国国家生物技术信息中心建库至2017年10月，收录的关于抗LGI1抗体相关脑炎的文献进行检索，并总结该病患儿的临床特征。本研究符合2013年修订的《世界医学协会赫尔辛基宣言》。

①患儿1，男性，8岁，为目前报道年龄最小的儿童抗LGI1抗体相关脑炎患儿。主诉"夜间睡眠减少50 d"，临床主要表现为夜间睡眠减少伴兴奋，家族史及生长发育史未见异常，神经系统体格检查未见异常。头颅MRI提示左侧海马病变。脑脊液及血清抗LGI1抗体检测结果呈阳性。该例患儿接受静脉注射用人免疫球蛋白(IVIG)及泼尼松治疗后，夜间睡眠减少伴兴奋及头颅MRI较前明显好转。②患儿2，男性，15岁，主诉"发作性抽搐7 d"，临床主要表现为抽搐，形式为复杂部分性发作和部分性发作继发强直-阵挛，不伴记忆力下降及认知、精神、睡眠及运动障碍，血清抗LGI1-IgG抗体检测结果呈阳性(1∶100)，该例患儿接受IVIG及左乙拉西坦片治疗后，随访1^＋年的结果显示无神经、精神症状，学习成绩良好。③设定检索策略进行相关文献检索的结果显示，仅检索到3篇国外文献报道涉及3例年龄<18岁抗LGI1抗体相关脑炎患儿(14、15、17岁)。其中，14岁患儿的症状为近期记忆力障碍、精神行为异常，为目前公开报道的最小年龄抗LGI1抗体相关脑炎患儿。

本研究8岁患儿为目前国内外报道的年龄最小抗LGI1抗体相关脑炎患儿。儿童抗LGI1抗体相关脑炎可单纯以睡眠障碍或癫痫发作起病，头颅MRI表现具有典型特征，采取IVIG和糖皮质激素治疗疗效良好。

To analyze the clinical features of the leucine-rich glioma-inactivated 1 protein (LGI1) antibody related encephalitis in children and review the literature.

From March to November 2016, in Department of Neurology of Children′s Hospital, Capital Institute of Pediatrics, and Beijing Tian Tan Hospital, two cases of LGI1 antibody related encephalitis children (patient 1 and patient 2) were chosen as research objects. Case data of 2 children were analyzed and clinical features of disease were summarized. With the following key words of ＂leucine rich glioma inactivation 1 protein＂ and ＂immunity encephalitis＂ in Chinese, ＂LGI1＂ ＂leucine-rich glioma-inactivated 1 protein＂ and ＂autoimmune diseases of the nervous system＂ in English, literature of anti-LGI1 antibody related encephalitis were searched from WanFang data knowledge service platform, the medical knowledge network, PubMed database, and National Center for Biotechnology Information, literature retrieval time start from the establishment of database to October 2017, and clinical features of searched literature were summarized. This study was consistent with the World Medical Association Declaration of Helsinki revised in 2013.

①Patient 1 was a 8 years old boy, who was the youngest child currently reported to have anti-LGI1 antibody related encephalitis. Chief complaint was ＂night sleep reduced for 50 days＂, the mainly clinical manifestations were night sleep reduced and accompanied by night excitement, no abnormal family history and growth history, no abnormal neurophysical examination. Head MRI showed left hippocampal lesions. Cerebrospinal fluid and serum anti-LGI1 antibody were positive. After treated by intravenous injection of human immunoglobulins (IVIG) and prednisone, the clinical manifestations and head MRI of the child were improved obviously. ②Patient 2 was a 15 years old boy and chief complaint was＂paroxysmal seizures for 7 days＂, the main clinical manifestation was tic, tic form were complex partial seizure and tonic-clonus secondary to partial seizures, not accompanied with memory decline, cognitive disorders, mental disorders, sleep disorders and movement disorders, and serum anti LGI1-IgG antibody positive (1: 100). This child was treated with IVIG and levetiracetam tablets, follow-up for more than 1 year without nerve system and mental symptoms, and with good academic performance. ③ With the retrieval strategy we setting up, literature retrieval results showed that only 3 foreign literature reported anti-LGI1 antibody related encephalitis involving 3 cases of patients of age under 18 years old as 14, 15, 17 years old, respectively. The clinical symptoms of 14 years old anti-LGI1 antibody related encephalitis child were recent memory disorder and mental behavior disorder, who was the youngest child with anti-LGI1 antibody related encephalitis currently reported in public.

The 8-year-old child in this study was the youngest child with anti-LGI1 antibody related encephalitis currently reported at home and abroad. The clinical manifestations of children with anti-LGI1 antibody related encephalitis can be simply onset with sleep disorder or seizure disorder, and head MRI performance has the typical characteristics, the effect of IVIG and glucocorticoid therapy is good.