抗LGi1抗体阳性边缘系统脑炎叠加Hashimoto脑炎临床特点分析并文献复习(附1例报告)

Clinical analysis of anti-leucine-rich glioma inactivated-1 protein positive limbic encephalitis co-existing with Hashimoto encephalitis

目的总结抗LGi1抗体阳性边缘系统脑炎的临床与影像学特点,加深对于本病的认识。方法报告于2013年11月25日就诊于北京积水潭医院神经内科门诊后收住院确诊的1例抗LGi1抗体阳性边缘系统脑炎患者的整个临床发病及诊治转归过程,对于患者的临床资料、影像学、电生理资料进行分析总结。结果患者为中年女性,亚急性起病,主要临床表现包括极其频繁发作的面部肩部不适及面部抽动、频率稍低的发作性幻嗅、逐渐出现的记忆力下降、精神淡漠等认知及精神障碍;头部MRI在T2及Flair序列上见到了双侧颞叶内侧、海马区的高信号病灶,脑电图见到多导广泛的高幅慢波及棘慢或尖慢综合波等癫痫样放电;血及脑脊液LGi1抗体均阳性,同时血甲状腺抗过氧化物酶抗体及甲状腺抗球蛋白抗体均明显增高,提示叠加了Hashimoto脑炎,此外尚合并部分其他自身抗体的阳性;在接受了大剂量甲基强的松龙冲击并逐渐减量的治疗方案后,患者病情迅速缓解好转,随访1 y未再复发。结论抗LGi1抗体阳性边缘系统脑炎作为一种罕见的以损伤边缘系统为主的自身免疫性脑炎,在边缘系统脑炎基本表现的基础上,有其特殊的临床变现。同时还可以合并其他自身免疫相关脑炎及自身抗体阳性,尽早明确诊断并给予一线的免疫治疗可明显改善患者预后。

Objective To study the clinical and imaging features of anti-leucine-rich glioma inactivated-1 protein positive（ anti-LGI1） limbic encephalitis. Methods By reviewing the clinical course,relative examination results and outcome of the disease,here we share a case of anti-LGI1 limbic encephalitis. Results The patient was a middle-aged female,suffering from sub-acute onset of frequent facio-brachial discomforts,orofacial dyskinesia,olfactory hallucination,confusion,memory deficits,and psychiatric disturbances. MRI demonstrated hyper-intensities within both medial temporal lobe structures and bilateral hippocampus on T2 and FLAIR sequences. EEG showed slow wave activity with relatively high amplitude or epileptic form discharges. Anti-LGI1 antibody was identified in serum and CSF. Meanwhile,several other autoimmune antibodies including antithyroid antibodies were positive which indicated co-existing Hashimoto encephalitis. Treatment with methylprednisolone achieved satisfactory improvement. And her clinical conditions remained stable on the 1-year-follow-up.Conclusion Anti-LGI1 limbic encephalitis has unique clinical features that is distinguishable from other classic limbic encephalitis due to autoimmune damage of limbic areas. Some cases could also be found accompanied by other autoimmune encephalitis or other autoimmune antibodies. Early first-line immunotherapies may improve clinical prognosis.