自身免疫性脑炎临床特征初探  

Preliminary Study on Clinical Features of Autoimmune Encephalitis

作　　者：王晓华[1] 史向松 潘合跃 王守勇[1] 徐建洋[1] WANG Xiao-hua;SHI Xiang-song;PAN He-yue;WANG Shou-yong;XU Jian-yang(Department of Neurology,Huai'an Third People's Hospital,Jiangsu 223001,China)

机构地区：[1]淮安市第三人民医院神经科,江苏淮安223001

出　　处：《神经损伤与功能重建》2020年第7期380-383,共4页Neural Injury and Functional Reconstruction

摘　　要：目的:探讨自身免疫性脑炎(AE)的临床特征、治疗及预后。方法:对7例AE患者的临床表现、实验室检查、治疗及预后等临床资料进行回顾性分析。结果:7例AE患者中,4例为抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,其中2例伴畸胎瘤;2例为抗富含亮氨酸胶质瘤失活蛋白1(LGI1)脑炎;1例为抗γ-氨基丁酸B型受体(GABABR)脑炎。以精神行为异常(5/7)、癫痫发作(4/7)、意识清晰度下降(4/7)、面-臂肌张力障碍发作(FBDs)(1/7)、近事记忆力下降(2/7)为主要临床表现。2例颅脑MRI检查异常。2例脑脊液(CSF)抗NMDAR抗体阳性,2例CSF抗LGI1抗体阳性,1例CSF抗GABABR抗体阳性。2例血清抗NMDAR抗体阳性,1例血清抗GABABR抗体阳性。7例经治疗后4例基本恢复,2例遗留癫痫发作(1例伴精神行为异常),复发1例。结论:以急性发作的精神行为异常、癫痫发作及FBDs为主要临床表现的患者要警惕AE可能。AE临床治疗以激素、免疫球蛋白及手术(伴有畸胎瘤患者)治疗为主,早期诊疗多数预后良好。Objective:To investigate the clinical features,treatment,and prognosis of autoimmune encephalitis(AE).Methods:The clinical data,laboratory examination,treatment,and prognosis of 7 patients with AE were retrospectively analyzed.Results:Of the 7 AE cases,4 were anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis,and 2 of these were associated with teratomas;2 cases were anti-leucine-rich glioma inactivating protein 1(LGI1)encephalitis;1 case was anti-gamma-aminobutyric acid type B receptor(GABABR)encephalitis.The main clinical manifestations were abnormal mental behavior(5/7),seizure(4/7),decreased consciousness(4/7),faciobrachial dystonia seizures(1/7),and recent-memory loss(2/7).Brain MRI examination was abnormal in 2 cases.Cerebrospinal fluid AE-related antibody detection found 2 anti-NMDAR antibody positive cases,2 anti-LGI1 antibody positive cases,and 1 anti-GABABR antibody positive case.Serum AE-related antibody detection revealed 2 anti-NMDAR antibody positive cases and 1 anti-GABABR antibody positive case.After all patients received treatment,4 patients recovered,2 exhibited seizures(1 of whom also experienced abnormal mental behavior),and 1 relapsed.Conclusion:Patients with acute episodes of mental behavior abnormalities,seizures,and faciobrachial dystonia seizures should be alert to AE.Hormones,immunoglobulins,and surgery(in patients with teratoma)are the main clinical treatments.Early diagnosis and treatment generally produce a good prognosis.

关 键 词：自身免疫性脑炎 N-甲基-D-天冬氨酸受体 富含亮氨酸胶质瘤失活蛋白1 γ-氨基丁酸B型受体 

分 类 号：R741[医药卫生—神经病学与精神病学] R741.041[医药卫生—临床医学]