Analysis of Small Cell Lung Cancer with Paraneoplastic Limbic Encephalitis

: 【中文题目：小细胞肺癌合并副肿瘤性边缘叶脑炎病例
分析】 【中文摘要：背景与目的 本研究旨在探讨小细胞肺癌（small cell lung cancer, SCLC）合并副肿瘤性边缘叶脑炎（paraneoplastic limbic encephalitis, PLE）的诊断及治疗要点。方法 收集北京协和医院1980年1月-2017年5月收治的15例SCLC合并PLE患者的、临床资料，并分析其症状、实验室检查数据并随访其预后。结果 PLE是较为罕见的疾病类型，在SCLC中的发病率约为0.842%，该数据可能因误诊、漏诊而被低估；中年吸烟男性患者为高发人群，肿瘤-淋巴结-转移（tumor-node-metastasis, TNM）分期多偏晚；神经系统症状多为典型的边缘叶脑炎症状，包括不同程度的短期记忆力丧失、癫痫发作及不同程度的精神异常；神经系统症状多先于肿瘤发现或呼吸系统症状出现，发病到确诊中位时间约2个月；实验室检查中多有血清抗体（抗Hu、GABA-R-Ab）、脑脊液、头磁共振成像（magnetic resonance imaging, MRI）及脑电图异常；影像学尤其是计算机断层扫描（computed tomography, CT）是筛查原发肿瘤较好的手段，病理确诊主要依靠支气管镜；针对原发肿瘤的治疗比免疫治疗能够更有效地缓解神经系统症状。结论 副肿瘤性边缘叶脑炎是一种较为罕见的由恶性肿瘤导致的远隔性神经系统副肿瘤综合征，常以边缘神经系统症状为特征性表现，多数与肺癌（尤其是SCLC）相关，其神经系统异常多早于肿瘤诊断，早期诊断及针对原发肿瘤的治疗将提高获益。
】 【中文关键词：肺肿瘤；副肿瘤性边缘叶脑炎；副肿瘤综合征】. METHODS: The clinical data of 15 patients with SCLC combined with PLE from January 1980 to May 2017 were collected from Beijing Union Hospital. Their symptoms and laboratory data were analyzed and the prognosis of the patients was followed. RESULTS: PLE is a rare disease, the incidence rate in SCLC is about 0.842%. The data may be underestimated because of misdiagnose or missed diagnosis; High incidence crowd of the disease is the middle-aged male smoker, the tumor-node-metastasis (TNM) stages of them are later than others; Typical neurological symptoms include varying degrees of short-term memory loss, seizures and varying degrees of mental disorders; neurological symptoms usually occur before the onset of cancer or respiratory symptoms appear, an average of about 2 months be taken from onset to diagnosis; Serum antibody (anti-Hu, GABA-R-Ab), cerebrospinal fluid, head magnetic resonance imaging (MRI) and electroencephalogram (EEG) of the patients has abnormalities; Videography, especially computed tomography (CT) is a good means of screening the primary tumor, pathology diagnosis mainly rely on bronchoscopy; The treatment of primary tumors can be more effective in alleviating the nervous system symptoms than immunotherapy. CONCLUSIONS: Paraneoplastic limbic encephalitis is a rare paraneoplastic syndrome in nervous system caused by malignant neoplasms often characterized by facial neurological symptoms. The disease are usually associated with lung cancer (especially SCLC). Its nervous system symptoms occur earlier than the tumor diagnosis. Early diagnosis and treatment for primary tumors will increase the benefit.