文章摘要
王晓华,史向松,潘合跃,王守勇,徐建洋.自身免疫性脑炎临床特征初探[J].神经损伤功能重建,2020,15(7):380-383
自身免疫性脑炎临床特征初探
Preliminary Study on Clinical Features of Autoimmune Encephalitis
  
DOI:
中文关键词: 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
英文关键词: autoimmune encephalitis  N-methyl-D-aspartate receptor  leucine-rich glioma inactivating protein 1  gamma-aminobutyric acid type B receptor
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作者单位
王晓华,史向松,潘合跃,王守勇,徐建洋 淮安市第三人民医 院神经科 
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中文摘要:
      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。
英文摘要:
      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.
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