摘要
目的总结不同抗神经细胞抗体阳性的自身免疫性脑炎(AE)患者临床特点及预后。方法回顾性分析2020年1月至2022年6月新乡医学院第一附属医院神经内科诊治的12例不同抗神经细胞抗体阳性AE患者的临床表现、影像学、抗体检测及治疗,采用改良Rankin量表(mRS)对预后进行评价。结果男性患者4例,女性患者8例,年龄18~72岁,抗N-甲基-D-天冬氨酸(NMDAR)抗体脑炎3例,抗接触蛋白关联蛋白2(CASPR2)抗体脑炎2例,抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体脑炎5例,抗谷氨酸脱羧酶65(GAD65)抗体脑炎1例,1例为抗γ氨基丁酸B型受体(GABABR)抗体阳性的抗NMDAR脑炎,同时合并抗Hu抗体IgG阳性。7例表现为癫痫发作,3例为精神症状,2例为面臂肌张力障碍,1例表现为头痛、记忆力下降,1例为腹部抽动。9例影像学头颅MRI异常,累及双侧海马、杏仁体、基底节、大脑半球、脑膜。10例血清及脑脊液AE抗体一致、1例不一致、1例仅脑脊液抗体阳性。12例经免疫治疗均好转,其中1例甲泼尼龙冲击、足量人免疫球蛋白治疗效果差,给予二线免疫药物利妥昔单抗治疗,症状无改善,给予升级免疫药物托珠单抗治疗后症状逐渐好转;1例停药后复发。12例患者预后均较好(mRS≤2分)。结论AE的抗体分类多,临床表现以癫痫发作较常见,影像学以海马受累为主,早期诊断,尽早启动免疫治疗,预后好。
Objective To summarize the clinical characteristics and prognosis of different autoimmune encephalitis(AE)patients with positive anti neural cell antibodies.Methods From January 2020 to June 2022,the clinical manifestations,imaging,antibody detection and treatment of 12 patients with different anti neurocyte antibody positive AE diagnosed and treated in Neurology were analyzed retrospectively.The modified Rankin scale(mRS)was used to evaluate the prognosis.Results There were 4 men and 8 women,aged 18-72 years old,3 cases of anti anti-N-methyl-D-aspartate receptor(NMDAR)antibody encephalitis,2 cases of anti contactin-associated protein 2(CASPR2)antibody encephalitis,5 cases of anti leucine rich glioma inactivated 1(LGI1)antibody encephalitis,1 case of anti glutamic acid decarboxylase 65(GAD65)antibody encephalitis,and 1 case of anti GAD65 antibody encephalitis γ anti NMDAR encephalitis withγaminobutyric acid-B receptor(GABABR)antibody positivity,combined with anti Hu antibody IgG positivity.Seven patients presented with Seizure,three with mental symptoms,two with facial arm dystonia,one with headache and memory loss,and one with abdominal twitch.MRI abnormalities of 9 imaging heads involved bilateral hippocampus,amygdala,basal ganglia,cerebral hemisphere and meninges.Ten cases had consistent serum and cerebrospinal fluid AE antibodies,1 case was inconsistent,and 1 case was only positive for cerebrospinal fluid antibodies.After immunotherapy,12 cases were all improved,of which 1 case was treated with methylprednisolone shock and sufficient human immunoglobulin,and the effect was poor.After treatment with the second-line immune drug rituximab,the symptoms did not improve.After treatment with the upgraded immune drug tocilizumab,the symptoms gradually improved.One case recurred after discontinuing the medication.The prognosis of all 12 patients was good(mRS≤2 points).Conclusion There are many types of antibodies in AE.Seizure is the most common clinical manifestation.Hippocampal involvement is the main imaging feature.Early diagnosis and early initiation of immunotherapy can lead to a good prognosis.
作者
张建龙
关肖霄
赵帅
葛晓敏
李合华
ZHANG Jianlong;GUAN Xiaoxiao;ZHAO Shuai;GE Xiaomin;LI Hehua(Department of Neurology,the First Affiliated Hospital of Xinxiang Medical University,Xinxiang 453100,China)
出处
《河南医学研究》
CAS
2024年第13期2323-2328,共6页
Henan Medical Research
基金
2021年度新乡医学院第一附属医院青年培育基金项目B类资助项目(ON-2021-B07)。
