摘要
目的:报道3例以面-臂肌张力障碍发作(faciobrachial dystonic seizure,FBDS)为主要临床表现的抗富亮氨酸胶质瘤失活1蛋白(leucine-rich glioma inactivated 1,LGI1)抗体相关边缘性脑炎(limbic encephalitis,LE)患者的诊治经过,并对相关文献进行复习。方法:对上海交通大学医学院附属仁济医院收治的3例以FBDS为主要临床表现的抗LGI1抗体相关LE病例的临床特征、辅助检查、治疗及预后资料进行回顾性分析,并对相关文献进行复习。结果:3例抗LGI1抗体相关LE病例均以FBDS为主要临床表现之一,通过腰椎穿刺脑脊液检查、神经影像学检查以及相关血液和脑脊液自身免疫抗体检测等确诊为抗LGI1抗体相关LE。3例患者经免疫治疗后,症状均明显好转,其中1例由于发生迟发性硬膜下血肿而转科至神经外科接受手术治疗。结论:FBDS为抗LGI1抗体相关LE的特征性临床表现,早期识别有助于该病诊断,免疫治疗可有效提高患者的预后。
Objective: To report the diagnosis and treatment of 3 cases of limbic encephalitis(LE) related to antibodies against leucine-rich glioma inactivated 1(LGI1) protein with faciobrachial dystonic seizure(FBDS) as the main clinical manifestation, and review the related literatures.Methods: The information of clinical features, laboratory examinations, imaging examinations, treatment and prognosis of 3 cases diagnosed of anti-LGI1 LE with FBDS as the main clinical manifestation admitted in Renji Hospital, Shanghai Jiao Tong University School of Medicine was reported and analyzed, and the related literature review was performed.Results: In these 3 cases,FBDS was the very early main clinical symptom, and they were diagnosed of anti-LGI1 LE by lumber test, brain magnetic resonance imaging(MRI) and autoimmune antibodies examination. The symptoms were relieved by immunotherapy, whereas one case underwent operation due to late subdural hematoma.Conclusion: FBDS is a characteristic clinical manifestation of anti-LGI1 LE, early identification is helpful for the diagnosis of this disease, and immunotherapy could effectively improve the prognosis.
出处
《神经病学与神经康复学杂志》
2017年第4期186-196,共11页
Journal of Neurology and Neurorehabilitation
