摘要
目的分析双重抗神经元抗体阳性自身免疫性脑炎(AE)患者的临床特征、抗体检测结果、脑电图及影像学表现、治疗和随访情况,提高对该病的认识。方法收集2012年12月至2019年11月在宁波市医疗中心李惠利医院诊治的双重抗神经元抗体阳性的AE患者8例,分析患者临床特征、抗体检测结果、脑电图及影像学表现、治疗和随访情况。结果8例患者中男3例,女5例;年龄35~72岁。3例为抗N-甲基-D-天冬氨酸受体抗体合并抗γ-氨基丁酸B型受体(GABABR)抗体阳性,2例为抗富含亮氨酸胶质瘤失活蛋白1抗体合并抗接触蛋白相关蛋白2抗体阳性,2例为抗GABABR合并抗Hu抗体阳性,1例为抗GABABR合并抗Yo抗体阳性。8例患者的主要临床表现均为癫痫发作、认知功能障碍、精神行为异常等边缘性脑炎表现,例4合并小脑性共济失调,例8合并肌强直、肌痛等莫旺综合征表现。3例患者合并肺癌,1例患者怀疑为肺癌。所有患者均接受激素冲击和免疫球蛋白治疗。确诊或怀疑肿瘤的4例患者预后较差,其中3例(例3~5)随访期间死亡,例6患者出院3个月后遗留记忆力下降。未发现合并肿瘤的4例患者随访期间病情均显著缓解,预后较好。结论双重抗神经元抗体阳性的AE患者多以边缘性脑炎为主要临床表现,可有周围神经病变、小脑性共济失调等临床表现的叠加和变异。患者多合并肿瘤,此类患者预后差。临床上需重视对双重抗神经元抗体阳性AE的诊治。
Objective To investigate the clinical characteristics of patients with dual anti-neuronal antibody positive autoimmune encephalitis(AE).Methods The clinical data of 8 patients with dual antibody positive AE admitted in Ningbo Medical Treatment Center Lihuili Hospital fromDecember 2012 to November 2019 were retrospectively analyzed.Results Among the 8 patients,3 were male and 5 were females,aged 35-72 years.3 cases were positive for NMDAR and GABABR antibody,2 cases were positive for LGI1 and CASPR2 antibody,2 cases were positive for GABABR and anti-Hu antibody,and 1 case was positive for GABABR and anti-Yo antibody.The main manifestations of all 8 patients were limbic encephalitis.Case 4 was accompanied by cerebellar ataxia,and case 8 was associated with Morvan syndrome presenting as myotonia and myalgia.3 patients had lung cancer,and 1 patient had suspected lung cancer.All patients were treated with hormones and immunoglobulins.In 4 patients with confirmed or suspected tumors,3 died during follow-up,and the remaining one had impairment of memory 3 months after discharge.Other 4 patients were found to have significant remission during follow-up.Conclusion The main clinical manifestations of patients with dual neuron antibody positive AE are limbic encephalitis,peripheral neuropathy and cerebellar ataxia.The patients are often complicated with tumors and for those patients the prognosis is poor.
作者
戴俊杰
石露
DAI Junjie;SHI Lu(Department of Neurology,Ningbo Medical Treatment Center Lihuili Hospital,Ningbo 315040,China)
出处
《浙江医学》
CAS
2020年第10期1025-1029,共5页
Zhejiang Medical Journal
基金
浙江省医药卫生科技计划项目(2019KY604)。
