电压门控钾离子通道复合物相关抗体脑炎7例分析

目的:探讨电压门控钾离子通道复合物(VGKC)相关抗体脑炎的临床特点及诊治。方法:回顾性分析VGKC相关抗体脑炎7例的临床资料。结果:患者发病前无前驱症状;出现癫痫发作6例,精神症状3例,认知障碍2例;伴睡眠障碍者4例,心慌、多汗1例,低钠血症者3例,面-肩臂肌张力障碍者1例。脑脊液富亮氨酸胶质瘤失活蛋白1(LGI-1)抗体阳性4例(其中2例同时血清LGI-1抗体阳性),血清LGI-1抗体阳性1例,脑脊液和血清接触蛋白相关蛋白2(Caspr2)抗体阳性1例,血清Caspr2抗体阳性1例。头MRI平扫表现为主要累及颞叶内侧、海马区伴或不伴基底节区T2和FLAIR序列高信号;视频脑电图监测异常3例。均未发现肿瘤。应用丙种球蛋白、甲泼尼龙冲击或二者联合治疗,随访3个月,好转6例,复发、放弃治疗1例。结论:VGKC相关抗体脑炎首发症状多为癫痫,LGI-1脑炎多伴有低钠血症、睡眠障碍,Caspr2脑炎多伴有自主神经功能障碍。头颅MRI不能鉴别脑炎的类型,脑电图无特征性的改变,脑脊液和血清自免脑抗体检测有助于确定诊断。多数病例早期免疫治疗有效,少数病例可复发。

Analysis of 7 Cases of VGKC-Complex Antibody-Associated Encephalitis

To analyze the clinical feature, diagnosis, and treatment of voltage-gated potassiumchannel complex (VGKC) antibodies-associated encephalitis. Methods: We retrospectively analyzed theclinical data of 7 patients with VGKC antibodies-associated encephalitis. Results: Patients showed noprodromal symptoms prior to onset. There were 6 cases with seizure, 3 cases with psychiatric symptoms, and 2cases with cognitive decline. There were 4 cases with sleep disorder, 1 case with palpitation or hyperhidrosis, 3cases with hyponatremia, and 1 case with face, shoulder, and arm dystonia. There were 4 patients that werepositive for cerebrospinal fluid (CSF) leucine-rich glioma-inactivated protein 1 (LGI-1) antibodies; among these,2 patients were also positive for serum LGI-1 antibodies. One patient was positive for serum LGI-1 antibodies; 1patient was positive for CSF and serum contactin-associated protein 2 (Caspr2) antibodies; 1 patient was positivefor serum Caspr2 antibodies. Brain magnetic resonance imaging scans exhibited abnormalities mainly in thetemporal lobe, hippocampus, and basal ganglia with or without high-intensity T2 and FLAIR sequence signals.Electroencephalographs showed abnormality in 3 patients. No tumor was detected by screening. Gammaglobulin, methylprednisolone, or a combination of both were used as treatment. After a 3-month follow up, 6patients showed improvement and 1 patient relapsed and abandoned treatment. Conclusion: Onset of VGKCantibodies-associated encephalitis is characterized by seizure. LGI-1 encephalitis is characterized byhyponatremia and sleep disorder. Caspr2 encephalitis is characterized by autonomic dysfunction. Brain MRI isunable to distinguish between encephalitis types. Electroencephalographs show no characteristic changes.Detection of CSF and serum autoimmune antibodies is conducive to confirming diagnosis. Early immunotherapyis effective in most cases, and relapse occurs in few cases.