14例抗代谢型谷氨酸受体5脑炎患者临床资料分析  

Clinical analysis of 14 patients with anti-metabotropic glutamate receptor 5 encephalitis

作　　者：冯基伦 程璇[1] 张海峰[1] 连亚军[1] Feng Jilun;Cheng Xuan;Zhang Haifeng;Lian Yajun(Department of Neurology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,China)

机构地区：[1]郑州大学第一附属医院神经内科,郑州450000

出　　处：《中华神经医学杂志》2022年第2期172-175,共4页Chinese Journal of Neuromedicine

基　　金：国家自然科学基金面上项目(81771397)。

摘　　要：目的总结抗代谢型谷氨酸受体5(mGluR5)脑炎的临床特点及影像学表现,以提高临床医生对该病的认识。方法报道郑州大学第一附属医院神经内科2020年12月收治的1例抗mGluR5脑炎患者的临床资料,并结合国内外数据库中已报道的13例单一抗mGluR5脑炎患者资料进行汇总分析。结果14例患者主要症状为精神行为异常(13/14)、认知障碍(11/14)、睡眠障碍(9/14)、癫痫发作(8/14)、意识水平下降(6/14)及运动障碍(4/14)。4例患者头部MRI异常,包括T2像上右侧颞叶内侧高信号、双侧脑桥上部高信号,FLAIR像上双侧顶枕叶后部皮质高信号及T2/FLAIR像上双侧额叶、右侧枕叶/小脑高信号。6例患霍奇金淋巴瘤。治疗包括免疫治疗和抗肿瘤治疗。14例患者中共有3例复发,使用免疫疗法后症状仍有显著改善。除1例患者院内死亡外,末次随访时(中位时间20个月),6例完全恢复,7例部分恢复。结论抗mGluR5脑炎症状不局限于边缘系统,存在复发可能,免疫治疗效果良好;患者多合并霍奇金淋巴瘤。Objective To summarize the clinical and imaging characteristics of anti-metabotropic glutamate receptor 5(mGluR5)encephalitis,and improve clinicians'understanding of this encephalitis.Methods The clinical data of one patient with anti-mGluR5 encephalitis admitted to our hospital from December 2020 were analyzed,and the data of 13 patients with anti-mGluR5 encephalitis reported in domestic and foreign databases were summarized and analyzed.Results The main symptoms of 14 patients with anti-mGluR5 encephalitis included mental and behavior disorder(13/14),cognitive dysfunction(11/14),sleep dysfunction(9/14),seizures(8/14),decreased level of consciousness(6/14)and motor disorders(4/14).Four patients had abnormal brain MRI,including hyperintensity in the right mesial temporal lobe on T2 sequences,hyperintensity in the bilateral upper pons on T2 sequences,hyperintensity in the bilateral posterior parietal-occipital cortex on FLAIR sequences,and hyperintensity in the bilateral frontal lobes,right occipital lobe and cerebellum on T2/FLAIR sequences.Six patients had Hodgkin's lymphoma.Treatments included immunotherapy and oncologic therapy.Relapse was noted in 3 of the 14 patients,and symptoms improved significantly after immunotherapy.Except for one patient who died in hospital,6 patients showed complete recovery and 7 patients showed partial recovery at the last follow-up(median 20 months).Conclusions Anti-mGluR5 encephalitis is rare autoimmune encephalitis whose symptoms are not limited to the limbic system,and relapse may occur;immunotherapy has good effects;Hodgkin's lymphoma is often associated in these patients.

关 键 词：抗代谢型谷氨酸受体5脑炎 自身免疫性脑炎 霍奇金淋巴瘤 

分 类 号：R742.9[医药卫生—神经病学与精神病学]