14例抗Hu抗体阳性神经系统副肿瘤综合征临床特征分析

Clinical features of 14 cases of anti-Hu antibody-positive paraneoplastic neurologic syndrome

目的探讨抗Hu抗体阳性神经系统副肿瘤综合征(PNS)的临床特征。方法收集2016年1月至2020年9月郑州大学第一附属医院收治的抗Hu抗体阳性的确诊PNS的患者,均依据2021年副肿瘤综合征更新诊断标准及PNS诊断评分系统(PNS-Care Score)进行诊断。患者均随访至2021年8月,分析其临床表现、治疗及预后。结果共收入14例PNS患者,男8例、女6例,年龄42~74岁,平均(58.93±9.50)岁。14例PNS患者中表现为周围神经病9例(64.3%),其中7例(50.0%)为感觉运动神经病、2例(14.3%)为感觉神经元病,表现为边缘性脑炎和快速进展性小脑综合征各2例(14.3%)、脑干脑炎1例(7.1%);合并小细胞肺癌11例(78.6%),乳腺癌、胸腺瘤及B细胞淋巴瘤各1例(7.1%)。14例中11例(78.6%)在肿瘤确诊之前出现神经系统症状,延迟诊断15 d至14个月,中位延迟时间为4.00(2.00,6.00)个月。14例PNS患者中,8例接受肿瘤治疗,8例接受免疫治疗,其中甲泼尼龙冲击6例、丙种球蛋白治疗2例,3例仅接受对症治疗;3例病情好转,11例死亡。截至2021年8月,14例PNS患者的中位生存时间为12.00(4.75,16.50)个月。结论本组抗Hu抗体阳性的PNS临床表现多样,以周围神经病最多见,均合并肿瘤,以小细胞肺癌为多,总体预后差。早期诊断并及时治疗原发肿瘤可能是改善PNS预后的关键。

Objective To investigate the clinical features of anti-Hu antibody-positive paraneoplastic neurologic syndrome(PNS).Methods Patients with anti-Hu antibody positive and confirmed PNS admitted to the First Affiliated Hospital of Zhengzhou University from January 2016 to September 2020 were collected and the diagnosis was made according to the updated diagnostic criteria of paranoplastic syndrome 2021 and PNS-Care Score.All patients were followed up until August 2021,and their clinical manifestations,treatment and prognosis were analyzed.Results A total of 14 patients were collected,including 8 males and 6 females,aged from 42 to 74 years,with an average age of 58.93±9.50 years.9 cases(64.3%)presented with peripheral neuropathy,including 2 cases(14.3%)of sensory neuronopathy,7 cases(50.0%)of sensorimotor neuropathy;2 cases(14.3%)of limbic encephalitis,2 cases(14.3%)of rapidly progressive cerebellar syndrome,and 1 cases(7.1%)of brainstem encephalitis.11 cases(78.6%)complicated with small cell lung cancer,1(7.1%)breast cancer,1(7.1%)thymoma and 1(7.1%)B-cell lymphoma.11 of the 14 patients(78.6%)developed neurological symptoms before tumor diagnosis,delaying diagnosis by 15 days to 14 months,and the median delay was 4.00(2.00,6.00)months.Among the 14 patients with PNS,8 patients received tumor therapy and 8 patients received immunotherapy,including 6 patients with methylprednisolone shock therapy,2 patients with gamma globulin therapy,and 3 patients only received symptomatic therapy.Three patients improved and 11 died.As of August 2021,the median survival time of 14 patients with PNS was 12.00(4.75,16.50)months.Conclusions In this group,the clinical manifestations of anti-Hu antibody positive PNS had various clinical manifestations,most of which were peripheral neuropathy,mostly complicated with small cell lung cancer,and the overall prognosis was poor.Early diagnosis and timely treatment of the primary tumor may be the key to improve the prognosis of PNS.