Chinese Medical E-ournals Database

Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition) ›› 2018, Vol. 14 ›› Issue (03): 305 -310. doi: 10.3877/cma.j.issn.1673-5250.2018.03.009

Special Issue:

Original Article

Two cases of children with leucine-rich glioma-inactivated 1 protein antibody related encephalitis and literature review

Jianzhao Zhang1, Cuijuan Xu1, Xiushan Ge1, Ping Zheng1, Haitao Ren2, Xiaoqiu Shao3, Qian Chen1,(), Hongzhi Guan2,()   

  1. 1. Department of Neurology, Children′s Hospital, Capital Institute of Pediatrics, Beijing 100020, China
    2. Department of Neurology, Peking Union Medical College Hospital of Chinese Academy of Medical Science, Beijing 100730, China
    3. Department of Neurology, Beijing Tian Tan Hospital, Capital Medical University, Beijing 100050, China
  • Received:2018-02-21 Revised:2018-05-10 Published:2018-06-01
  • Corresponding author: Qian Chen, Hongzhi Guan
  • About author:
    Corresponding authors: Chen Qian, Email:
    Guan Hongzhi, Email:
Objective

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

Method

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.

Results

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.

Conclusions

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.

图1 患儿1,8岁,男性,血清抗富亮氨酸胶质瘤失活1蛋白抗体间接免疫荧光试验呈阳性(绿色荧光)
图2 患儿1,8岁,男性,头颅MRI显示患儿左侧海马区异常信号,符合边缘性脑炎;经免疫治疗后异常信号范围明显缩小(图2A:治疗前T2WI;图2B:治疗前T2Flair成像;图2C:治疗后2周T2WI;图2D:治疗后2周T2Flair成像)
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