自身免疫性脑炎的临床特征分析
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南京医科大学第一附属医院

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国家自然科学基金项目(面上项目,重点项目,重大项目)


Clinical characteristics of autoimmune encephalitis
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    摘要:

    摘要: 目的:总结和分析50例自身免疫性脑炎(autoimmune encephalitis,AE)患者的临床表现、脑电图特点、实验室及影像学特点等。 方法:回顾性分析2019年11月至2023年3月期间,在江苏省人民医院神经内科确诊为AE的临床资料。根据不同抗体类型进行分组,比较各抗体脑炎的基线资料特征;并根据首发症状是否为癫痫发作分类两组,比较组间的基线资料特征。 结果:共纳入研究50例患者,男性占比62%(31/50),女性38%(19/50),平均发病年龄为46±17.89岁。最常见的首发症状为癫痫发作(56%,28/50),最常见的临床表现为癫痫发作(70%,35/50),抗富亮氨酸胶质瘤失活1蛋白(leucine-rich glioma inactivated 1 ,LGI1)抗体脑炎特征性临床特征为脸臂肌张力障碍性癫痫和低钠血症(P=0.033)。80%(40/50)的患者出现脑电图异常,表现为弥漫或/局灶性慢波或癫痫样放电。66%(33/50)的患者头颅磁共振异常,其中颞叶或海马异常63.64%(21/33)。首发症状为癫痫发作的患者男性占比较高(P =0.001)。 结论: AE临床特点多样,低钠血症和脸臂肌张力障碍性癫痫为抗LGI1抗体脑炎特征性症状。AE患者病程中出现全面强直-阵挛发作或癫痫持续状态,需警惕其缓解后持续发作或发展为慢性癫痫的风险。大部分AE患者脑电图异常,但缺乏特异性,表现为弥漫或/局灶性慢波或癫痫样放电。影像学检查大部分表现为颞叶异常信号。 关键词:自身免疫性脑炎;自身免疫性癫痫;脑电图

    Abstract:

    Abstract: Objective: To summarize and analyze the clinical manifestations, EEG features, laboratory and imaging features of 50 patients with autoimmune encephalitis. Methods: Clinical data of patients diagnosed with AE in the Department of Neurology of Jiangsu Provincial People's Hospital from November 2019 to March 2023 were retrospectively analyzed. Groups were grouped according to different antibody encephalitis, and the baseline data characteristics were compared among the groups. They were grouped according to whether the first symptom was seizure or not, and the baseline data characteristics between the two groups were compared. Results: A total of 50 patients were included in the study, with males accounting for 62% (31/50) and females 38% (19/50). The average age of onset was 46±17.89 years old. The most common first symptom was epileptic seizure (56%, 28/50), the most common clinical presentation was epileptic seizure (70%, 35/50), and the characteristic clinical features of encephalitis with anti-leucine-rich glioma inactivated 1 protein (LGI 1) antibody were facial arm dystonia epilepsy and hyponatraemia(P =0.033). 80% (40/50) of patients developed EEG abnormalities that presented as diffuse or/focal slow wave or epileptiform discharges. 66% (33/50) of patients had abnormal cranial MRI, and 63.64% (21/33) had abnormal temporal lobe or hippocampus. The first symptom of epilepsy was higher in males (P =0.001). Conclusion: The clinical characteristics of AE are varied. Hyponatremia and facial arm dystonia epilepsy are the characteristic symptoms of anti-LGI 1 antibody encephalitis. Patients with AE who develop general tonic-clonic seizures or persistent epileptic states during the course of the disease need to be aware of the risk of continuing seizures or developing chronic epilepsy after remission. The majority of patients with AE have abnormal electroencephalogram, but lack of specificity, with diffuse or/focal slow wave or epileptiform discharges. Most of the imaging findings showed abnormal temporal lobe signals. Key words: autoimmune encephalitis; Autoimmune epilepsy; Electroencephalography;

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  • 收稿日期:2023-01-17
  • 最后修改日期:2023-06-30
  • 录用日期:2023-10-20
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