摘要
目的探讨富亮氨酸胶质瘤失活1蛋白（leucine-rich glioma inactivated 1,LGI1）自身抗体相关边缘性脑炎的临床特点、诊断及治疗。方法对2013年7月~2015年6月临床怀疑边缘性脑炎（LE）患者的血清和脑脊液标本,采用间接免疫荧光法,检测血及脑脊液中的抗LGI1抗体及其他边缘性脑炎相关抗体。对其中抗LGI1抗体阳性的8例患者资料进行登记整理并进行随访。结果共8例抗LGI1抗体阳性的LE患者,平均年龄52.6岁。以急性或亚急性起病,100%有面-臂肌张力障碍发作（faciobrachial dystonic seizures,FBDS）,6例患者（75%）伴有其他类型癫痫,5例患者（62.5%）出现认知功能障碍。8例患者血清抗LGI1抗体100%阳性,脑脊液抗LGI1抗体5例（62.5%）阳性;血清及脑脊液其他边缘性脑炎抗体8例（100%）均为阴性;7例（87.5%）患者有低钠血症。5例患者MRI示海马、颞叶（双侧或单侧）出现异常信号,3例头部MRI未见明显异常,1例行PET检查,脑实质内未见异常。除1例未行脑电检查外,其余病例脑电均有异常,其中有3例24 h脑电提示发作间期双颞区见较多中幅欠规则尖慢波及尖化θ波,左右不同步出现。6例接受免疫治疗,结果显示丙种球蛋白治疗联合激素治疗优于单独激素治疗,未接受免疫治疗患者也有自发缓解趋势。结论 LGI1-Ab相关LE有较特异的临床表现,如FBDS、低钠血症和认知功能障碍等;免疫治疗效果好。
Objective To further characterize the clinical phenotypes, diagnosis and therapy in Leucine-rich glioma inactivated-1 （ LGI1 ） antibody associated limbic encephalitis. Methods The anti-neuronal antibodies were checked by in- direct immunofluorescence test of serum or CSF. Then we analyzed clinical characteristics of 8 patients with LGII antibod- ies, and outcomes according to therapeutic strategies. Results LGI1 antibodies were identified in 8 patients ranging in age from 40 to 73 years （median ,52.6 years）. All patients experienced faciobrachial dystonic seizures （FBDS） , among the 8 patients,6 （75%） presented with seizures,while 5 patients （62.5%） showed cognitive dysfunction, mainly deficits in memory and abnormal behavior. The positive of anti-LGI1 antibody of＂ serum was 100% ,while it was 62.5% in CSF. The paraneoplastic antibodies （anti-Hu,anti-Yo,anti-Ri,anti-Ma2,-CV2/CRMPS, and Amphiphysin） were not detected in any of the 8 patients. The 87.5% patients had hyponatremia at the early days. MRI scans were performed in all patient,with 5 patients （62.5%） showing increased signals on MRI fluid-attenuated inversion recovery or T2 sequences. Except one was not taken EEG,the other 7 patients exhibited abnormalities in their EEGs. FBDS were controlled more effectively with im- munotherapy than anti-epileptic drugs. The patients treated with steroids alone were more likely to relapse and less favorable outcomes than those treated with steroids and intravenous immunoglobulin. Conclusion LGI1-ab associated LE has u- nique clinical manifestations. The respond to immunotherapy, especially combination of steroids with intravenous immuno- globulin, is good.
出处
《中风与神经疾病杂志》
CAS
北大核心
2015年第9期783-787,共5页
Journal of Apoplexy and Nervous Diseases
