抗CASPR2抗体相关脑炎癫痫发作的临床特征

Clinical Features of Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis with Seizure

目的分析抗接触蛋白相关蛋白-2(CASPR2)抗体相关脑炎癫痫发作的临床特征。方法对2018年9月至2020年11月郑州大学第五附属医院及郑州大学第一附属医院神经内科收治的7例抗CASPR2抗体相关脑炎癫痫发作患者的临床表现、辅助检查、治疗及预后进行回顾分析。结果7例患者年龄10~72岁,中位年龄62岁,其中5例男性,2例女性。癫痫发作类型中复杂部分性发作(CPS)5例(71.43%),全面性强直-阵挛发作(GTCS)4例(57.14%),未见其特征性发作表现,4例(57.14%)出现边缘系统症状,5例(71.43%)合并自主神经功能障碍。血清学未见特异性改变,脑脊液检查可出现蛋白、淋巴细胞水平升高,血清抗体检测敏感度高于脑脊液。2例(28.57%)出现头颅磁共振成像(MRI)异常,3例(42.86%)脑电图可见异常。所有患者均接受癫痫药物、免疫治疗,6例好转。随访1例复发,2例死亡。结论以癫痫发作为主要症状的脑炎患者需考虑抗CASPR2抗体相关脑炎的可能,及时进行抗体检测明确诊断,早期给予抗癫痫、免疫治疗,一般预后较好。

Objective To analyze the clinical features of anti-contactin-associated protein-like 2(CASPR2)antibody-associated autoimmune encephalitis with seizure.Methods The clinical manifestations,auxiliary examination results,treatment and prognosis of 7 patients with anti-CASPR2 antibody-associated autoimmune encephalitis with seizure who were admitted to the Department of Neurology of the Fifth Affiliated Hospital of Zhengzhou University and the First Affiliated Hospital of Zhengzhou University from September 2018 to November 2020 were retrospectively analyzed.Results The age of 7 patients ranged 10 to 72 years old.The median age was 62 years old.There were 5 males and 2 females among them.There were 5 cases(71.43%)of complex partial seizure(CPS)and 4 cases(57.14%)of generalized tonic-clonic seizure(GTCS).No characteristic seizure was found.There were 4 patients(57.14%)with symptoms of limbic system and 5 patients(71.43%)with autonomic neuropathy.No specific changes were found in serology.The levels of protein and lymphocyte increased in cerebrospinal fluid.The sensitivity of antibody detection in serum was higher than that in cerebrospinal fluid.Magnetic resonance imaging(MRI)of the brain showed abnormal signal in 2 cases(28.57%),and 3 cases(42.86%)showed abnormal electroencephalogram.All the patients received antiepileptic therapy and immunotherapy,and 6 cases improved after treatment.The follow up results showed 1 case recurred and 2 cases died.Conclusion Encephalitis patients with epilepsy as the main symptom should be suspected of anti-CASPR2 antibody-associated autoimmune encephalitis.Antibody detection is carried out in time to make a clear diagnosis.The patients who are treated with early anti-epilepsy and immunotherapy generally have good prognosis.