14例副肿瘤性周围神经病临床特征分析  

Clinical characteristics of 14 patients with paraneoplastic peripheral neuropathy

作　　者：刘举 潘佳丽 杨晓艳[1] 魏芷桦 陈敏 刘洪波[1] LIU Ju;PAN Jiali;YANG Xiaoyan;WEI Zhihua;CHEN Min;LIU Hongbo(Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院神经内科,河南郑州450052

出　　处：《肿瘤基础与临床》2022年第4期327-331,共5页journal of basic and clinical oncology

基　　金：国家自然科学基金资助项目(U2004128);河南省医学科技攻关计划(联合共建)项目(LHGJ20190086)。

摘　　要：目的探讨副肿瘤性周围神经病(PPN)的临床特征。方法基于2021年副肿瘤性神经综合征(PNS)诊断标准,回顾性分析郑州大学第一附属医院2015年1月至2021年6月收治的PPN患者的临床表现、PNS抗体、肌电图、治疗及预后等。结果纳入14例PPN患者,8例为确诊PNS,3例为很可能,3例为可能。男9例,女5例。发病年龄37~71(55.9±11.3)岁。临床表型:5例为感觉神经病,9例为感觉运动神经病,均亚急性或慢性起病,以肢体麻木无力、疼痛为主要症状。PNS抗体:5例抗Hu抗体阳性,1例抗Hu、GD1a及GM2抗体阳性,1例抗Hu、CV2抗体阳性,1例抗Hu、SOX1抗体阳性,3例抗Amphiphsin抗体阳性,2例抗Yo阳性,1例抗CV2阳性。合并肿瘤:6例患小细胞肺癌(SCLC),1例患淋巴瘤,1例患乳腺癌,6例未发现肿瘤。肌电图示:5例为感觉神经病变,9例为感觉运动神经病变;8例以轴突损害为主,6例以脱髓鞘病变为主。治疗及预后:6例接受化疗,1例免疫抑制治疗,1例激素冲击治疗,3例死亡,4例无缓解或加重,5例缓解。结论PPN患者表现为感觉神经病或感觉运动神经病,以抗Hu抗体阳性者和SCLC最多见,可采用免疫抑制剂治疗,早期治疗原发肿瘤,少数可缓解症状,但总体预后不佳。Objective To investigate the clinical features of paraneoplastic peripheral neuropathy(PPN).Methods Based on the 2021 updated diagnostic criteria of paraneoplastic neurological syndrome(PNS),the clinical manifestations,PNS antibodies,electromyogram,treatment and prognosis of patients with PPN diagnosed and treated in the First Affiliated Hospital of Zhengzhou University from January 2015 to June 2021 were retrospectively analyzed.Results Fourteen patients with PPN were included,8 patients with confirmed PNS,3 patients with probable PNS,and 3 patients with possible PNS.There were 9 males and 5 females with an average age of(55.9±11.3)years,ranging from 37 to 71 years.Clinical phenotype:5 patients were sensory neuropathy and 9 patients were sensorimotor neuropathy,with subacute or chronic onset,and the main symptoms were numbness,weakness and pain of limbs.PNS antibodies:5 patients were positive for anti-HU,1 patient for anti-HU,GD1a and GM2,1 patient patients for anti-HU and CV2,1 patient for anti-HU and SOX1,3 patients for anti-Amphiphsin,2 patients for anti-Yo,and 1 patient for anti-CV2.Complicated tumor:6 patients with small cell lung cancer(SCLC),1 patient with lymphoma,1 patient with breast cancer,and 6 patients without tumor.Electromyography showed sensory neuropathy in 5 patients and sensorimotor neuropathy in 9 patients;8 patients exhibited mainly axonal lesions,and 6 patients mainly demyelinating lesions.Treatment and prognosis:6 patients received chemotherapy,1 patient received immunosuppressive therapy,and 1 patient received high-dose hormone therapy;3 patients died,4 patients had no remission or exacerbated,and 5 patients had remission.Conclusion PPN patients present with sensory neuropathy or sensorimotor neuropathy,most of which are found in patients with anti-HU antibody positive and SCLC.Immunosuppressive therapy or early treatment of primary tumor can be applied,and a few symptoms can be relieved,but the overall prognosis is poor.

关 键 词：副肿瘤性周围神经病 副肿瘤性神经综合征 小细胞肺癌 抗Hu抗体 临床特征 

分 类 号：R747.9[医药卫生—神经病学与精神病学]