抗两性蛋白抗体相关副肿瘤性脑干脑炎伴食管神经内分泌癌1例  

Anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma: a case report

作　　者：王妍颖 毛晨晖[1] 杨璐 贾丛伟[2] 周良锐 朱文佳[3] 关鸿志[1] 朱以诚[1] 崔丽英[1] Yanying Wang;Chenhui Mao;Lu Yang;Congwei Jia;Liangrui Zhou;Wenjia Zhu;Hongzhi Guan;Yicheng Zhu;Liying Cui(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Nuclear Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院神经科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院病理科,北京100730 [3]中国医学科学院北京协和医学院北京协和医院核医学科,北京100730

出　　处：《中华神经科杂志》2022年第11期1298-1302,共5页Chinese Journal of Neurology

基　　金：中国医学科学院医学与健康科技创新工程(CIFMS)临床与转化医学研究基金(2020-I2M-C&T-B-010)。

摘　　要：副肿瘤神经综合征(PNS)是恶性肿瘤继发的自身免疫反应所致的一组异质性疾病, 可累及神经系统的任何部分。抗两性蛋白抗体为PNS高风险抗体之一, 常伴发小细胞肺癌及乳腺癌, 而肺外神经内分泌癌少见。我们报道1例伴发食管神经内分泌癌的抗两性蛋白抗体相关副肿瘤性脑干脑炎患者。通过氟脱氧葡萄糖正电子发射体层摄影发现肿瘤, 由胃镜活组织检查病理证实。患者经人免疫球蛋白和糖皮质激素治疗后, 神经系统症状部分改善。最终预后与伴发肿瘤密切相关。Paraneoplastic neurological syndromes(PNS)are heterogeneous disorders caused by autoimmune responses of cancer,which can affect any part of the nervous system.Anti-amphiphysin antibody is one of the high-risk PNS antibodies,which is usually associated with small cell lung cancer and breast cancer.However,extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody.A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported.The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy.The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids.However,the disease prognosis is closely related to the accompanying tumor.

关 键 词：副肿瘤综合征 神经系统 两性蛋白 神经内分泌癌 脑干脑炎 

分 类 号：R739.41[医药卫生—肿瘤]