摘要
目的总结抗神经元表面抗原抗体(NSAbs)相关自身免疫性脑炎(AE)患者的临床特点。方法回顾性分析首都医科大学附属北京地坛医院2012-05—2017-04诊治的7例抗NSAbs相关AE患者的临床特征及实验室检查结果特点,并进行相关文献复习。结果 7例患者中男5例,女2例,年龄15~77岁。3例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎;2例抗γ氨基丁酸B型受体(GABABR)脑炎,其中1例合并抗Hu抗体阳性;2例抗富亮氨酸胶质瘤失活1蛋白(LGI1)脑炎。4例患者以癫痫为突出或惟一表现,1例患者仅表现为头痛、发热,2例患者临床表现符合经典边缘性脑炎。1例合并肺癌(抗GABABR脑炎合并抗Hu抗体阳性患者)。6例脑电图检查可见癫痫波发放,1例仅表现为头痛、发热的抗NMDAR脑炎患者脑电图未见异常。3例头MRI检查可见异常信号。7例患者经免疫治疗均好转,例1有复发。结论抗NSAbs相关AE可以癫痫为突出或惟一的临床表现,或以头痛、发热为惟一的临床症状。特异性抗NSAbs检测阳性可协助明确诊断。
Objective To explore the clinical features of autoimmune encephalitis(AE)associated with neuronal surface antibodies(NSAbs).Methods Seven patients of AE associated with NSAbs hospitalized from May 2012 to April 2017 were retrospectively reviewed.The clinical manifestations and investigations were summarized,and related literatures were reviewed.Results Five male and 2 female patients,aging from 15 to 77 years old were included in this study,including 3 cases of anti-NMDAR encephalitis,2 cases of anti-GABA BR encephalitis(anti-Hu antibody was positive in 1 case),and 2 cases of anti-LGI1 encephalitis.Epilepsy was prominent or the only clinical manifestation in 4 patients,1 patient presented with only headache and fever,and 2 patients were consistent with classical limbic encephalitis.Lung cancer was found in a patient of anti-GABA BR encephalitis with positive anti-Hu antibody.Six patients showed abnormal EEG.Only 1 patient showed a normal EEG.Abnormal findings in cranial MRI were found in 3 cases.Immunosuppressive therapy was effective in the 7 patients.Relapse occurred in 1 case.Conclusions AE associated with NSAbs can manifest as epilepsy,or with headache and fever as the only clinical symptoms.Positive NSAbs can help confirm the diagnosis.
作者
李务荣
高俊华
姜美娟
伍文清
黄宇明
许东梅
LI Wurong;GAO Junhua;JIANG Meijuan;WU Wenqing;HUANG Yuming;XU Dongmei(Department of Neurology,Beijing Ditan Hospital,Capital Medical University,Beijing 100015,China)
出处
《中国神经免疫学和神经病学杂志》
CAS
北大核心
2018年第1期32-36,共5页
Chinese Journal of Neuroimmunology and Neurology
