目的:分析常见自身免疫性脑炎的临床特点,评价抗体对局灶性癫痫体征和症状(ACES)评分的敏感性。方法:回顾性分析2015年8月至2020年12月郑州大学第一附属医院收治的242例自身免疫性脑炎(AE)患者的临床资料。ACES评分的六项(认知症状,行为改变,自主神经症状,言语问题,自身免疫性疾病,时域MRI高强度)在具有完整临床数据的患者中进行筛查。结果:(1)总体上,242名患者被纳入,147例抗N-甲基-D-天冬氨酸受体脑炎,抗γ-氨基丁酸B型(GABA-B)受体脑炎47例,和48例抗富含亮氨酸的胶质瘤失活蛋白1(LGI1)脑炎。最常见的临床症状是认知障碍(77%),行为变化(79%),和缉获量(71%)。总的来说,129例(54%)合并自主神经功能障碍,如胃肠动力障碍,窦性心动过速,和中央通气不足。12名患者患有自身免疫性疾病,其中大多数是甲状腺疾病。(2)127例临床资料完整的患者评估ACES评分,126例(126/127,99.2%)等于或>2分,1例(1/127,0.8%)<2分。解释:(1)认知障碍,异常行为,癫痫发作是AE和自主神经症状的最常见表现。甲状腺疾病是AE中最常见的自身免疫性疾病。临床上,对于疑似AE的患者,增加甲状腺功能和风湿病的知识和测试是必要的。(2)ACES评分很简单,有效,和易于操作的分数,对大多数疑似AE患者具有一定的筛查价值。
Objective: To analyze the clinical features of common autoimmune encephalitis and evaluate the sensitivity of antibodies contributing to focal epilepsy signs and symptoms (ACES) score. Methods: Collecting and analyzing the data of 242 patients with autoimmune encephalitis (AE) diagnosed in the First Affiliated Hospital of Zhengzhou University from August 2015 to December 2020 in this retrospective study. The six items of the ACES score (cognitive symptoms, behavioral changes, autonomic symptoms, speech problems, autoimmune diseases, temporal MRI hyperintensities) were screened in patients with complete clinical data. Results: (1) In total, 242 patients were included, with 147 cases of anti-N-methyl-D-aspartate receptor encephalitis, 47 cases of anti-γ-aminobutyric acid type B (GABA-B) receptor encephalitis, and 48 cases of anti-leucine-rich glioma inactivating protein 1 (LGI1) encephalitis. The most common clinical symptoms are cognitive impairment (77%), behavioral changes (79%), and seizures (71%). In total, 129 cases (54%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, sinus tachycardia, and central hypoventilation. Twelve patients had autoimmune diseases, most of which were of thyroid diseases. (2) One hundred and twenty-seven patients with complete clinical data evaluated ACES score, 126 cases of whom (126/127, 99.2%) were equal to or >2 points, 1 case (1/127, 0.8%) was of <2 points. Interpretation: (1) Cognitive impairment, abnormal behavior, and seizures are the most common manifestations of AE and autonomic symptoms. Thyroid disease is the most autoimmune disease in AE. Clinically, for patients of suspected AE, increasing the knowledge and testing of thyroid function and rheumatism is necessary. (2) ACES score is a simple, effective, and easy-to-operate score, with a certain screening value for most patients suspected of AE.