抗代谢型谷氨酸受体1抗体相关性脑炎1例并文献复习  

A case report of mGluR1 antibody related encephalitis and literature review

作　　者：桂梦翠 林婧 葛慧珍 卜碧涛[1] GUI Mengcui;LIN Jing;GE Huizhen;BU Bitao(不详;Department of Neurology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan Hubei 430030,China)

机构地区：[1]华中科技大学同济医学院附属同济医院神经科,430030

出　　处：《中国神经免疫学和神经病学杂志》2024年第5期358-363,共6页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的探讨抗代谢型谷氨酸受体1(mGluR1)抗体相关性脑炎的临床特征及预后,以提高临床医生对本病的认识。方法报道一例抗mGluR1抗体相关脑炎患者的临床资料,并结合文献报道的34例抗mGluR1抗体相关脑炎患者的临床资料进行回顾性分析。结果患者为38岁男性,主要表现为头晕、共济失调2个月余,脑脊液蛋白、IgG指数轻度增高,头颅MRI平扫+增强检查结果正常,血清及脑脊液mGluR1-IgG滴度均为1∶100,诊断为抗mGluR1抗体相关脑炎,患者经过大剂量糖皮质激素、丙种球蛋白、利妥昔单抗等治疗后明显好转。35例抗mGluR1抗体相关脑炎患者年龄(48.3±20.3)岁,临床主要表现为小脑共济失调(33例)、认知功能下降(11例)。8例(22.9%)患者合并有肿瘤性疾病,所有患者血清或/和脑脊液mGluR1抗体阳性。头颅MRI主要表现为小脑半球或蚓部、小脑幕异常。85.7%(30/35)的患者接受免疫治疗,其中11例患者使用2种免疫治疗,11例使用3种及以上的免疫治疗。随访结果显示,21例(60.0%)患者好转,4例(11.4%)患者好转后复发,7例(20.0%)患者病情稳定,2例(5.7%)患者无明显变化,1例(2.9%)患者死亡。结论抗mGluR1抗体相关脑炎主要表现为小脑共济失调,血清或脑脊液抗mGluR1抗体检测有助于诊断,大多数患者经糖皮质激素联合丙种球蛋白或血浆置换或免疫抑制剂或利妥昔单抗治疗后好转,少部分患者有复发且预后差。Objective To explore the clinical manifestations,treatment and prognosis of encephalitis with metabotropic glutamate receptors 1(mGluR1)antibody in order to improve clinicians’understanding of this disease.Methods The clinical data of a patient with mGluR1 antibody was reported,and a retrospective analysis was carried out based on 34 patients with mGluR1 antibody in the literature.Results A 38-year-old man mainly manifested as dizzy and ataxia for about 2 months.The cerebrospinal fluid(CSF)protein and IgG index were slightly elevated,while head MRI scan and enhanced examination showed no criminal lesions.The serum and CSF mGluR1 IgG titers were both 1∶100.Diagnosis was made as anti-mGluR1 antibody related encephalitis.Symptoms were significantly relieved after methylprednisolone pulse,intravenous immunoglobulin and rituximab therapy.Including our case,35 cases have been reported.The average age of these patients was(48.3±20.3)years old.The main clinical symptoms included ataxia(33 cases)and decreased cognitive function(11 cases).Eight patients(22.9%)were concomitant with tumor,and all patients’serum and/or cerebrospinal fluid anti-mGluR1 antibodies was positive.85.7%(30/35)of the patients received immunotherapy,of which 11 patients received 2 immunotherapies,and 11 patients received 3 or more immunotherapies.At the end of follow-up,21 patients(60.0%)improved,4 patients(11.4%)relapsed,7 patients(20.0%)remained stable,1 patient(5.7%)remained unchanged and 1 patient(2.9%)died.Conclusions Anti-mGluR1antibody related encephalitis mainly manifests cerebellar ataxia.Serum or CSF anti-mGluR1 antibody testing is helpful for diagnosis.Most patients improve after treated with glucocorticoids combined with immunosuppressants or rituximab,while a small number of patients have recurrence and poor prognosis.

关 键 词：受体 亲代谢性谷氨酸盐 抗mGluR1抗体 自身免疫性脑炎 共济失调 临床特征 预后 

分 类 号：R742.9[医药卫生—神经病学与精神病学]