抗Hu抗体相关副肿瘤周围神经病三例临床特点分析  

Paraneoplastic neuropathy associated with anti-Hu antibody:report of three cases

作　　者：汪伟[1] 汪仁斌[1] 张伟赫[1] 佟杰[2] 孙少杰[1] 段晓慧[1] 刘尊敬[1] 焦劲松[1] 彭丹涛[1] WANG Wei;WANG Renbin;ZHANG Weihe;TONG Jie;SUN Shaojie;DUAN Xiaohui;LIU Zunjing;JIAO Jinsong;PENG Dantao(Department of Neurology,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]中日友好医院神经科,100029 [2]中日友好医院病理科,100029

出　　处：《中国神经免疫学和神经病学杂志》2019年第5期352-356,共5页Chinese Journal of Neuroimmunology and Neurology

基　　金：国家自然科学基金青年科学基金项目(81701131)

摘　　要：目的分析3例抗 Hu抗体相关副肿瘤周围神经病的临床特点。方法回顾性分析2015年7月至2017年7月于中日医院神经科诊治的3例抗 Hu抗体相关副肿瘤周围神经病患者的临床表现、实验室检查、影像学及神经电生理特点。结果 3例患者中男1例,女2例,年龄分别为66、58、59岁。患者主要临床表现为肢体疼痛(3例)、体位性低血压(2例)与麻痹性肠梗阻(1例)、步态不稳和共济失调(1例),1例脊髓 MRI检查可见脊髓后索病变,2例胸部CT检查发现占位性病变;3例腰穿脑脊液常规、生化检查结果正常范围,血清及脑脊液抗 Hu抗体均为阳性;神经电生理检查结果显示感觉神经动作电位(SNAP)波幅下降为著伴运动纤维受累,皮肤交感反应测定2例未引出波形,另1例潜伏期延长。病程早期,针对肿瘤治疗或未发现肿瘤患者行免疫调节治疗可部分缓解周围神经症状。结论抗 Hu抗体相关副肿瘤周围神经病以感觉纤维受累及自主神经病变为著,伴有运动纤维受累,血清及脑脊液 Hu抗体检测有助于诊断,肺部肿瘤筛查及早期治疗可部分改善患者周围神经症状。Objective To analyze the clinical features of the patients who had peripheral neuropathy associated with anti-Hu antibody. Methods Three patients with paraneoplastic neuropathy associated with anti-Hu antibody who were admitted to China-Japan Friendship Hospital from July 2015 to July 2017 were enrolled.The patients clinical manifestations,laboratory tests,imaging and neurophysiological characteristics were analyzed retrospectively. Results 3 patients were identified,including two female and one male,aged at 66,58 and 59 years old.The clinical presentations were subacute painful neuropathy involving distal limbs (3 cases),orthostatic hypotension (2 cases),pseudoileus (1 case)and sensory ataxia (1 case).Abnormal T2 signal in the posterior cord was detected by spinal MRI in 1 case.Lung mass was demonstrated in 2 cases by chest CT study and tumor was confirmed in one case by biopsy.Cerebrospinal fluid (CSF)examination showed normal white blood cell counts and normal protein level in all the subjects.All cases were anti-Hu antibody positive in both serum and CSF samples.On neural electrophysiological examination,all cases had decreased sensory nerve action potential (SNAP)amplitude,with decreased motor nerve conduction velocity in 2 cases.All the patients had abnormal sympathetic skin response.The patient who had lung cancer received tumor therapy;one patient with negative PET-CT study had immunotherapy. Conclusions Neuropathy patients associated with anti-Hu antibodies commonly present as subacute painful sensory neuropathy and autonomic symptoms,anti-Hu antibody testing is helpful for the diagnosis.Lung tumor screen and early therapy could partly resolve the neuropathy symptoms.

关 键 词：副肿瘤性多发性神经病 副肿瘤综合征 神经系统 Hu抗体 痛性周围神经病 自主神经病 共济失调 

分 类 号：R745[医药卫生—神经病学与精神病学]