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中国抗γ-氨基丁酸B受体脑炎患者的临床特征与预后

Clinical features and outcomes of Chinese patients with anti-γ-aminobutyric acid B receptor encephalitis.

作者信息

Zhao Xiu-He, Yang Xue, Liu Xue-Wu, Wang Sheng-Jun

机构信息

Department of Neurology, Qilu Hospital, Shandong University, Jinan, Shandong 250012, P.R. China.

出版信息

Exp Ther Med. 2020 Jul;20(1):617-622. doi: 10.3892/etm.2020.8684. Epub 2020 Apr 23.

Abstract

Antibodies against γ-aminobutyric acid B (GABAB) receptor are associated with limbic encephalitis (LE). It is estimated that ~1/2 of patients with LE have small-cell lung cancer. The present study analyzed the specific GABAB receptor antibodies in serum and cerebrospinal fluid (CSF) samples of 12 patients. The clinical manifestations, therapy and outcome were retrospectively compared. The median onset age was 65.1 years and all patients presented with new-onset seizures. In total, 11 (91.6%) patients had memory deficits, 7 (58.3%) patients had psychiatric problems and 4 (33.3%) patients had a disturbance of consciousness. Furthermore, lung cancer was detected in 7 patients (58.3%) by CT scan. Lymphocytic pleocytosis and protein concentration elevation in CSF were detected in 3 (25%) and 4 (33.3%) patients, respectively. Furthermore, MRI scan results identified 4 (33.3%) patients with abnormalities in the mesial temporal region. The lung cancer tissues of 3 patients were positively stained for anti-GABAB receptor on immunohistochemistry. All patients received antiepileptic drugs and immunotherapy. In total, 3 patients with lung cancer were subjected to tumor resection. Those patients without cancer exhibited neurological improvement at the follow-up. The present results suggested that seizures and memory deficits were the major manifestations in Chinese patients with anti-GABAB receptor antibodies who were responsive to immunotherapy. The lung cancer tissues from patients with anti-GABAB receptor antibodies were positively stained for anti-GABAB receptor. Collectively, the present results suggested that patients with underlying lung cancer have a relatively poor prognosis.

摘要

抗γ-氨基丁酸B(GABAB)受体抗体与边缘叶脑炎(LE)相关。据估计,约1/2的LE患者患有小细胞肺癌。本研究分析了12例患者血清和脑脊液(CSF)样本中的特异性GABAB受体抗体。对其临床表现、治疗及预后进行回顾性比较。中位发病年龄为65.1岁,所有患者均出现新发癫痫。共有11例(91.6%)患者有记忆缺陷,7例(58.3%)患者有精神问题,4例(33.3%)患者有意识障碍。此外,通过CT扫描在7例患者(58.3%)中检测到肺癌。分别在3例(25%)和4例(33.3%)患者中检测到CSF淋巴细胞增多和蛋白浓度升高。此外,MRI扫描结果显示4例(33.3%)患者在内侧颞叶区域有异常。3例患者的肺癌组织在免疫组化中抗GABAB受体呈阳性染色。所有患者均接受抗癫痫药物和免疫治疗。共有3例肺癌患者接受了肿瘤切除。那些没有癌症的患者在随访时神经功能有改善。目前的结果表明,癫痫和记忆缺陷是对抗GABAB受体抗体且对免疫治疗有反应的中国患者的主要表现。抗GABAB受体抗体患者的肺癌组织抗GABAB受体呈阳性染色。总体而言,目前的结果表明,患有潜在肺癌的患者预后相对较差。

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