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目的:回顾性分析和研究衡阳地区48例自身免疫性脑炎(AE)患者的临床特征、影像学表现、实验室检验和脑电图结果、免疫治疗效果及预后。方法:收集2020年1月至2024年4月在南华大学附属第一医院、南华大学附属第二医院及南华大学附属南华医院住院的48例AE患者的临床资料，对年龄、性别、前驱症状、首发症状、主要临床表现、头颅MRI、脑电图、脑脊液常规生化、血清与脑脊液抗体检测结果及免疫治疗效果等相关资料进行回顾性分析。结果:48例患者中，男性和女性各24例，发病年龄中位数为50岁。临床表现方面，精神行为异常为最常见的首发症状，占37.5%(18/48)；其次是癫痫发作，占35.4%(17/48)；16.7%(8/48)的患者出现认知障碍；少见的首发症状有运动障碍、视物模糊、意识丧失等。神经影像方面，47.9%(23/48)的患者头颅MRI有异常信号，其中颞叶或海马异常信号占47.8%(11/23)，额顶部占39.1%(9/23)，枕部占17.4%(4/23)，基底节和丘脑分别占13.0%(3/23)和8.7%(2/23)。脑电图方面，33.3%(16/48)的患者显示不同程度的脑电图异常，表现为弥漫性慢波、局灶性慢波、癫痫样放电或快波增多等。实验室检验方面，60.4%(29/48)的患者有脑脊液常规生化异常，其中白细胞数升高(>10×10 6个/L)占33.3%(16/48)，蛋白升高(>0.45 g/L)占37.5%(18/48)，IgG升高占33.3%(16/48)。抗体检测方面，27例为抗体阳性，包括抗NMDAR抗体17例、抗CASPR2抗体3例、抗mGluR5抗体3例、抗DPPX抗体2例、抗GlyR抗体1例、抗GAD65抗体1例。治疗方面，33例患者在对症治疗基础上实施了免疫治疗，其中13例患者的临床症状好转。 结论:AE患者临床表现具有高度的异质性，典型首发临床表现为精神异常、癫痫发作、认知障碍及运动障碍，神经影像学改变主要在颞叶或海马，抗体检测可及时明确AE的诊断，早期免疫治疗是改善AE预后的关键。

Objective:To retrospectively analyze and study the clinical characteristics, imaging manifestations, laboratory tests, electroencephalography (EEG) results, immunotherapy efficacy, and prognosis of 48 patients with autoimmune encephalitis (AE) in the Hengyang area.Methods:Clinical data of 48 AE patients admitted to the First Affiliated Hospital, the Second Affiliated Hospital, and the Affiliated Nanhua Hospital, University of South China from January 2020 to April 2024 were collected. Retrospective analysis was conducted on age, gender, prodromal symptoms, initial symptoms, main clinical manifestations, cranial magnetic resonance imaging (MRI), electroencephalogram, cerebrospinal fluid routine biochemistry, serum and cerebrospinal fluid antibody detection results, and immunotherapy efficacy.Results:Among the 48 patients, there were 24 males and 24 females, with a median age of onset of 50 years. In terms of clinical manifestations, abnormal mental behavior is the most common initial symptom, accounting for 37.5%(18/48); The next was epileptic seizures, accounting for 35.4%(17/48); 16.7%(8/48) of patients experienced cognitive impairment; Rare initial symptoms included movement disorders, blurred vision, and loss of consciousness. In terms of neuroimaging, 47.9%(23/48) of patients had abnormal signals on cranial MRI, including 47.8%(11/23) of abnormal signals in the temporal lobe or hippocampus, 39.1%(9/23) in the frontal and parietal regions, 17.4%(4/23) in the occipital region, 13.0%(3/23) in the basal ganglia, and 8.7%(2/23) in the thalamus. In terms of EEG, 33.3%(16/48) of patients showed varying degrees of EEG abnormalities, manifested as diffuse slow waves, focal slow waves, epileptic like discharges, or increased fast waves. In terms of laboratory testing, 60.4%(29/48) of patients had routine biochemical abnormalities in cerebrospinal fluid, including elevated white blood cell count (>10×10 6 cells/L) in 33.3%(16/48), elevated protein (>0.45 g/L) in 37.5%(18/48), and elevated IgG in 33.3%(16/48). In terms of antibody testing, 27 cases were positive for antibodies, including 17 cases of N-methyl-D-aspartate receptor (NMDAR) antibody, 3 cases of anti-contactin associated protein 2 (CASPR2) antibody, 3 cases of anti-metabotropic glutamate receptors 5 (mGluR5) antibody, 2 cases of anti-dipeptidyl-peptidase-like protein (DPPX) antibody, 1 case of anti-GlyR antibody, and 1 case of anti-GAD65 antibody. In terms of treatment, 33 patients received immunotherapy on the basis of symptomatic treatment, and 13 of them showed improvement in clinical symptoms. Conclusions:The clinical manifestations of AE patients are highly heterogeneous, with typical initial clinical manifestations including mental abnormalities, epileptic seizures, cognitive and motor disorders. Neuroimaging changes are mainly in the temporal lobe or hippocampus, and antibody detection can timely confirm the diagnosis of AE. Early immunotherapy is the key to improving the prognosis of AE.