Li Weishuai, Wu Si, Meng Qingping, Zhang Xiaotian, Guo Yang, Cong Lin, Cong Shuyan, Zheng Dongming
Department of Neurology, Shengjing Hospital of China Medical University, Sanhao Street 36, Shenyang, 110004, Liaoning, China.
BMC Neurol. 2018 Jul 6;18(1):96. doi: 10.1186/s12883-018-1099-z.
Recently, most reports of Leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis are from Europe and the US, while the short term outcome and clinical characteristics of Chinese patients are rarely reported,we study the clinical manifestations, laboratory results and brain magnetic resonance images (MRI) of eight patients who were recently diagnosed with LGI1 antibody encephalitis in our hospital to improve the awareness and knowledge of this disease.
Eight patients (five males and three females; mean age, 63.4) with LGI1 antibody encephalitis who were diagnosed and treated in the Department of Neurology of Shengjing Hospital of China Medical University from September 2016 to June 2017 were recruited for the current study. Their general information, clinical manifestations, treatment regimens, and short-term prognoses were retrospectively analyzed, as were the results from MRI and laboratory findings.
Overall, patient symptoms included cognitive impairment, which manifested primarily as memory deficits (8/8), seizures (including faciobrachial dystonic seizure, (FBDS)) (8/8), psychiatric and behavioral disorders (7/8), sleep disorders (4/8), and autonomic abnormalities (3/8). Five patients also had abnormal findings on brain MRI, mainly involving the hippocampus, basal ganglia and insula. Hyponatremia occurred in six cases. All patients tested positive for LGI1 antibodies in their serum/cerebrospinal fluid (CSF)and patients were negative for tumors. Symptoms rapidly improved after treatment with immunoglobulin and/or steroid therapy. The patients were followed up for 4-13 months after discharge, and two patients relapsed.
Primary symptoms of LGI1 antibody encephalitis include memory impairments, seizures, FBDS, and mental and behavioral abnormalities. Increased titers of LGI1 antibodies are also present in the serum/CSF of patients. Patients often have hyponatremia, and MRIs show abnormalities in various brain regions. Finally, immunotherapy shows good efficacy and positive benefits, although patients may relapse in the short-term.
近期,大多数关于富含亮氨酸胶质瘤失活1(LGI1)抗体脑炎的报道来自欧美地区,而中国患者的短期预后及临床特征鲜有报道。我们研究了我院近期诊断为LGI1抗体脑炎的8例患者的临床表现、实验室检查结果及脑磁共振成像(MRI),以提高对该疾病的认识。
选取2016年9月至2017年6月在中国医科大学附属盛京医院神经内科确诊并治疗的8例LGI1抗体脑炎患者(5例男性,3例女性;平均年龄63.4岁)进行本研究。回顾性分析其一般资料、临床表现、治疗方案及短期预后,以及MRI和实验室检查结果。
总体而言,患者症状包括认知障碍,主要表现为记忆缺陷(8/8)、癫痫发作(包括面臂肌张力障碍性发作,FBDS)(8/8)、精神和行为障碍(7/8)、睡眠障碍(4/8)及自主神经功能异常(3/8)。5例患者脑MRI也有异常表现,主要累及海马、基底节和脑岛。6例患者出现低钠血症。所有患者血清/脑脊液(CSF)中LGI1抗体检测均为阳性,且患者肿瘤相关检查为阴性。经免疫球蛋白和/或类固醇治疗后症状迅速改善。患者出院后随访4 - 13个月,2例患者复发。
LGI1抗体脑炎的主要症状包括记忆障碍、癫痫发作、FBDS以及精神和行为异常。患者血清/CSF中LGI1抗体滴度也升高。患者常伴有低钠血症,MRI显示多个脑区异常。最后,免疫治疗显示出良好疗效和积极效果,尽管患者可能在短期内复发。