抗NMDAR及抗LGI1相关脑炎急性期癫痫发作、临床特征及短期预后的对照研究

Clinical, seizure features in acute phase and short-term prognosis of anti-NMDAR and anti-LGI1 encephalitis: a control study

目的回顾性对照研究抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)及抗富亮氨酸胶质瘤失活1蛋白(Leucinie-rich gliomain activated 1,LGI1)相关脑炎患者急性期癫痫发作、临床特征及短期预后,为临床早期诊断和治疗提供参考。方法连续纳入2018年1月—2020年6月于四川省人民医院神经内科就诊的抗NMDAR及抗LGI1相关脑炎患者,回顾分析患者一般信息、临床表现、急性期癫痫发作情况及发作类型,评估两种自身免疫性脑炎在急性期癫痫发作的特点与短期预后的差异。结果共纳入75例抗NMDAR相关脑炎及抗LGI1相关脑炎患者,其中男41例,女34例,平均年龄(32.8±17.9)岁,平均病程(1.8±1.1)个月,其中抗NMDAR和抗LGI1抗体阳性分别59例和16例。75例中56例(74.7%)在急性期出现了癫痫发作,56例癫痫发作患者中伴有意识障碍38例(67.8%)、自主神经功能障碍5例(8.9%)、氧合能力下降24例(42.9%)、入住神经内科重症监护病房(NICU) 20例(35.7%),与无癫痫发作组比较有统计学差异(P<0.05)。抗NMDAR脑炎在急性期癫痫发作中位数年龄为23岁,抗LGI1脑炎为56.5岁(P<0.05)。抗NMDAR脑炎和抗LGI1脑炎在急性期癫痫发作均以全面性发作为常见(55.9%vs.53.8%),抗NMDAR脑炎在急性期更多出现反复癫痫发作及癫痫持续状态(P<0.05)。在早期合理使用抗癫痫药物(AEDs)及抗免疫等对症支持治疗后,56例患者中70%出院时癫痫得到有效控制,3个月后随访,18例(32.1%)患者停用AEDs,而30例(53.5%)患者仍继续接受AEDs治疗,其中25例患者(44.6%)癫痫无发作。结论抗NMDAR相关脑炎及抗LGI1相关脑炎急性期癫痫发作风险均较高,伴有癫痫发作患者更容易出现意识障碍、氧合能力下降、入住NICU比例更高。抗NMDAR脑炎更常见于30岁左右年轻人群,抗LGI1脑炎更易于60岁左右发病。抗NMDAR脑炎患者更容易出现脑电图异常、平均住院天数更长,在急性期更容易出现癫痫反复发作与癫痫持续状态,及时诊断及干预治疗后,大部分患者癫痫发作能得到良好控制,急性期过后约1/3患者可停用AEDs。

Objective To retrospectively study the acute epileptic seizures of patients with anti-N-methyl-Daspartate receptor(anti-NMDAR)and anti-leucine-rich glioma-inactivated 1(anti-LGI1)encephalitis. The characteristics and short-term prognosis provide reference for early clinical diagnosis and treatment. Methods The patients with antiNMDAR and anti-LGI1 encephalitis who were admitted to the Department of Neurology of Sichuan Provincial People’s Hospital from January 2018 to June 2020 were continuously included. The general information, clinical manifestations,acute seizures and types of seizures were reviewed and analyzed.To evaluate the difference between the characteristics of two kinds of autoimmune encephalitis in the acute phase of seizures and the short-term prognosis. Results A total of 75 patients with anti-NMDAR encephalitis and anti-LGI1 encephalitis(41 males and 34 females) were included, of which average ages are(32.8±17.9)years, average courses are(1.8±1.1)months.59 and 16 are respectively positive for antiNMDAR and anti-LGI1 antibodies, respectively. Of the 75 cases, 56 cases(74.7%) had seizures in the acute phase. Among the 56 cases of seizures, 38 cases(67.8%) were accompanied by disturbances of consciousness, 5 cases(8.9%) of autonomic dysfunction, and 24 cases of decreased oxygenation capacity.(42.9%) and 20 cases(35.7%) who were admitted to NICU,there was a significant statistical difference compared with the seizure-free group(P<0.05). The median age of antiNMDAR encephalitis in the acute stage of seizures was 23 years, and that of anti-LGI1 encephalitis was 56.5 years(P<0.05). Anti-NMDAR encephalitis and anti-LGI1 encephalitis are common in the acute phase of epileptic seizures(55.9%vs.53.8%). Anti-NMDAR encephalitis has more frequent seizures and status epilepticus in the acute phase(P<0.05).After early and rational use of anti-epileptic drugs(AEDs) and immunotherapy and other symptomatic and supportive treatments, 70% of 56 patients were effectively controlled for seizure. Follow-up 3 months later, 18 patients(32.1%) stopped using anti-epileptic drugs(AEDs), While 30 patients(53.5%) continued to receive AEDs treatment, of which 25 patients(44.6%) had no seizures. Conclusion Both anti-NMDAR encephalitis and anti-LGI1 encephalitis have a higher risk of seizures in the acute phase. Patients with seizures are more likely to have disturbances in consciousness, decreased oxygenation capacity, and higher rates of admission to NICU. Anti-NMDAR encephalitis is more common in young people around 30 years old, and anti-LGI1 encephalitis is more likely to develop around 60 years old. Patients with anti-NMDAR encephalitis are more likely to have abnormal electroencephalograms, have a longer average hospital stay, and are more likely to have recurrent seizures and status epilepticus in the acute phase. After timely diagnosis and intervention treatment, most patients’ seizures can be well controlled. After the acute phase, AEDs can be withdrawed in one third of patients.