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242例自身免疫性脑炎的临床特征与早期识别

Clinical Features and Early Recognition of 242 Cases of Autoimmune Encephalitis.

作者信息

Yang Mu, Lian Yajun

机构信息

Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

出版信息

Front Neurol. 2022 Jan 13;12:803752. doi: 10.3389/fneur.2021.803752. eCollection 2021.

Abstract

To analyze the clinical features of common autoimmune encephalitis and evaluate the sensitivity of antibodies contributing to focal epilepsy signs and symptoms (ACES) score. 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. (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. (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.

摘要

分析常见自身免疫性脑炎的临床特征,并评估有助于局灶性癫痫体征和症状的抗体(ACES)评分的敏感性。在这项回顾性研究中,收集并分析2015年8月至2020年12月在郑州大学第一附属医院确诊的242例自身免疫性脑炎(AE)患者的数据。在具有完整临床资料的患者中筛选ACES评分的六项内容(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、颞叶磁共振成像高信号)。(1)共纳入242例患者,其中抗N-甲基-D-天冬氨酸受体脑炎147例,抗γ-氨基丁酸B型(GABA-B)受体脑炎47例,抗富含亮氨酸胶质瘤失活蛋白1(LGI1)脑炎48例。最常见的临床症状是认知障碍(77%)、行为改变(79%)和癫痫发作(71%)。共有129例(54%)合并自主神经功能障碍,如胃肠动力障碍、窦性心动过速和中枢性通气不足。12例患者患有自身免疫性疾病,其中大多数为甲状腺疾病。(2)127例具有完整临床资料的患者评估了ACES评分,其中126例(126/127,99.2%)等于或大于2分,1例(1/127,0.8%)小于2分。(1)认知障碍、行为异常和癫痫发作是AE最常见的表现及自主神经症状。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,增加对甲状腺功能和风湿病的认识及检测是必要的。(2)ACES评分是一种简单、有效且易于操作的评分,对大多数疑似AE的患者具有一定的筛查价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f419/8793487/a57c015c8488/fneur-12-803752-g0001.jpg

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