摘要
目的探讨合并抗接触蛋白相关蛋白-2(CASPR2)抗体阳性的抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床特点、治疗及预后。方法收集2019年12月至2020年12月南昌大学第二附属医院神经内科收治的抗CASPR2抗体阳性的抗NMDAR脑炎患者5例,分析患者的临床特征、实验室检查、影像学检查、治疗及预后等资料。结果5例患者均为男性,发病年龄为39~82岁,均急性起病,3例表现为精神行为异常、癫痫等边缘系统受累症状,2例表现为发热、头痛等边缘系统外症状。颅脑MRI检查结果异常2例,1例示左侧颞叶异常信号,另1例示缺血灶及软脑膜强化。3例接受糖皮质激素联合丙种球蛋白治疗,1例接受抗病毒治疗,预后均良好,1例接受对症治疗者死亡。结论本组抗CASPR2抗体阳性的抗NMDAR脑炎患者主要表现为抗NMDAR脑炎症状,部分抗NMDAR脑炎复发可能叠加抗CASPR2抗体的产生,此类患者对一线免疫治疗反应良好,预后尚佳。
Objective To investigate the clinical features,treatment,and prognosis of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis patients with positive anti-contact protein-associated protein-2(CASPR2)antibody.Methods A total of 5 patients with anti-NMDAR encephalitis combined with anti-CASPR2 antibody who were admitted to the Department of Neurology of the Second Affiliated Hospital of Nanchang University from December 2019 to December 2020 were collected.The clinical characteristics,laboratory examination,imaging examination,treatment,and prognosis of these patients were analyzed.Results Five patients were included,all male,aged from 39 to 82 years old,and all had an acute onset.Three patients presented with limbic system involvement symptoms such as mental behavior disorder and epilepsy,and 2 patients presented with extra-limbic system symptoms such as fever and headache.Brain MRI showed abnormal results in 2 cases,1 case showed abnormal signals in the left temporal lobe,and the other 1 showed ischemic lesions and leptomeningeal enhancement.Three patients received glucocorticoid combined with immunoglobulin,1 patient received antiviral therapy,and the prognosis was good.One patient died after symptomatic treatment.Conclusions In this group,anti-CASPR2 antibody positive anti-NMDAR encephalitis patients mainly presented with anti-NMDAR encephalitis symptoms.Some of the anti-NMDAR encephalitis may be superposed with anti-CASPR2 antibody production.Such patients responded well to first-line immunotherapy,with a good prognosis.
作者
田胜
吴兰香
郑合情
刘盼
刘明煦
余欣萍
谢永钢
吴伟
TIAN Sheng;WU Lanxiang;ZHENG Heqing;LIU Pan;LIU Mingxu;YU Xinping;XIE Yonggang;WU Wei(不详;Department of Neurology,the Second Affiliated Hospital of Nanchang University,Nanchang Jiangxi 330006,China)
出处
《中国神经免疫学和神经病学杂志》
CAS
北大核心
2022年第3期216-219,225,共5页
Chinese Journal of Neuroimmunology and Neurology
基金
国家自然科学基金资助项目(31860293)。
