摘要
目的通过对抗富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关性边缘叶脑炎患者的临床资料进行分析和总结,并复习相关文献,加强临床对该疾病的认识。方法回顾性选取2013年4月至2017年1月在首都医科大学附属北京友谊医院住院的确诊为LGI1抗体相关性边缘叶脑炎患者4例,总结其临床资料,包括临床表现、影像学结果、血液和脑脊液检测结果、治疗和转归、随访结果等。结果 4例患者中,男性3例,女性1例,均为中老年患者,均表现为明显认知减退,以近记忆减退和定向障碍为主;3例出现不自主运动或震颤等锥体外系症状;1例以癫痫发作起病,精神行为异常1例、虚构和异食症1例等。4例患者均无明显意识障碍和低通气表现;头部MRI检查以及PET-CT结果均提示海马或颞叶内侧异常信号;4例患者脑脊液抗LGI1抗体阳性;3例患者同时存在顽固性低钠血症和低钾血症,均为轻-中度,1例患者低钾血症甚至持续1年以上;1例同时合并血抗髓鞘少突胶质细胞糖蛋白抗体(MOG)抗体阳性;3例患者给予丙种球蛋白治疗,1例给予甲基强的松龙冲击治疗;4例患者均遗留有不同程度的认知减退,生活基本自理。1例出院后失访,3例随访1~3年,均无复发,1例出院3年后猝死。结论抗LGI1抗体相关性边缘叶脑炎多见于中老年人,以近记忆减退和定向障碍等认知减退为主要表现,部分合并有低钠血症和低钾血症,影像学显示海马和颞叶内侧受累,丙种球蛋白或激素等免疫治疗有效,但可遗留认知损害。
Objective To have a profound comprehension of anti-leucine-rich,glioma inactivated 1（ LGI1） protein antibody related limbic encephalitis via clinical analysis of 4 cases and literature review. Methods This was a retrospective analysis of 4 cases of anti-LGI1 related limbic encephalitis from April 2014 to January 2017,including their clinical manifestation,radiological features,laboratory examinations,treatment and prognosis,as well as following-up. Results Four middle to old aged patients,3 male and 1 female,were included in this study. All of the 4 patients had memory dificit especially ecmnesia,and disorientation. Extra-pyramidal features like tremor and involuntary movements were seen in 3 cases,seizure in 1 case,mental and behavior disturbance in 1 case,fiction and allotriophagy in 1 case. Disturbance of consciousness or hypopnea was negative. The Magnetic resonance image and PET-CT examination showed abnormal signals seen in hippocampus and mesial temporal lobe. Anti-leucine rich glioma 1 inactive protein antibodies were positive in the cerebral spinal fluid of all the patients,the blood test showed mild and intractable hypokalemia and hyponatremia,which could last for more than 1 year. For other antibodies,serum anti-myelin-oligodendrocyte glycoprotein（ MOG） antibody was positive in 1 case. Intravenous immunoglobulin（ IVIG） was used for 3 patients and methylprednisolone stosstherapy for 1 patient. Four patients had incomplete recovery when discharge,with persisting memory deficit. One patient was lost to follow-up,other 3 patients was followed-up for 1 - 3 years,no relapse occur. 1 patients sudden died 3 years after discharge. Conclusion Anti-LGI1 related limbic encephalitis is common in elderly person,memory dificit and disorientation is the main clinical manifestation,hypokalemia and hyponatremia may be coexist in some cases,abnormal signals can be seen in hippocampus and mesial temporal lobe when giving radiological examination. Intravenous immunoglobulin and/or methylprednisolone stosstherapy is effective,but the patients may have incomplete recovery,memory deficit is remained.
作者
脱厚珍
田泽龙
薛云
杜艺彤
韩燕飞
高丹
张丽燕
TUO Hou - zhen;TIAN Ze - long;XUE Yun(Department of Neurology, Beijing Friendship Hospital, Capital Medical Univer- sity, Beijing 100050, China;Department of Neurology, The Fourth Central Hospital in Tianjin, Tianjin 300143, China.)
出处
《临床和实验医学杂志》
2018年第11期1141-1144,共4页
Journal of Clinical and Experimental Medicine
基金
国家自然科学基金(编号:30772287
81241039)
北京市教委课题(编号:KM201610025016)
