神经系统副肿瘤综合征47例临床分析

Analysis of clinical data of 47 cases of paraneoplastic neurological syndrome

目的探讨神经系统副肿瘤综合征(PNS)的临床特征。方法对2018年5月至2021月12月中国科学技术大学第一附属医院神经内科收治的47例PNS患者的临床资料进行回顾性分析。结果47例PNS患者中男性26例,占55.3%,女性21例,占44.7%,发病年龄范围在29岁~75岁,中位发病年龄58岁。47例患者中有40例(85.1%)为慢性或者亚急性起病,其中有35例(74.5%)发现合并肿瘤,最常见为肺癌18例(51.4%),有25例(71.4%)神经系统症状在肿瘤发现之前出现。47例PNS患者共涵盖了9种临床综合征类型,28例(59.6%)表现为经典综合征,以亚急性小脑变性和边缘叶脑炎最为常见;19例(40.4%)表现为非经典综合征,以感觉运动性周围神经病最为常见。经典综合征患者以中枢神经系统受累为主,非经典综合征常见周围神经系统受累。共40例患者检测出合并神经副肿瘤抗体,其中特征性抗体阳性者共28例,抗Hu抗体检出率(21%)最高,其次为抗Yo抗体(14.9%)。其他非特征性抗体阳性者共12例,双重抗体阳性者3例。共41例患者接受了肿瘤和/或免疫治疗,其中有31例(31/41;75.6%)治疗后一个月临床症状得到缓解,接受治疗前和治疗后mRS评分的差异经Wilcoxon秩检验有统计学意义(P<0.05)。共有31例短期预后良好(31/47;66.0%),16例短期预后不良(16/47;34.0%)。将短期预后良好和不良的两组患者的临床资料进行比较分析,两亚组患者在是否接受肿瘤和/或治疗方面存在显著性差异(P<0.05)。结论PNS以男性患者更为常见且多在中老年发病,主要以亚急性或慢性起病,合并肿瘤以肺癌居多,临床表现形式复杂多样,神经副肿瘤抗体的检测对于PNS的诊断非常重要。对于明确诊断为PNS的患者,应尽早积极的进行肿瘤和/或免疫治疗以缓解患者的临床症状,改善患者的预后。

Objective To explore the clinical features of paraneoplastic neurological syndrome(PNS).Methods A retrospective analysis was conducted on the clinical data of 47 patients,who were diagnosed with PNS and were admitted to the Department of Neurology,the First Affiliated Hospital of University of Science and Technology of China from May 2018 to December 2021.Results Among the 47 PNS patients,26 were male,accounting for 55.3%,and 21 were female,accounting for 44.7%.40(85.1%)of 47 patients had chronic or subacute oneset.The scope of age of onset was from 29 years old to 75 years old,and the medium age of onset was 58 years old.Combination tumors were found in 35(74.5%)of 47 patients,including 18 cases of lung cancers,accounting for 51.4%,and 25(71.4%)patients had neurological symptoms before tumor discovery.47 PNS patients covered a total of 9 clinical syndrome types,28(59.6%)patients showed classic syndrome,with subacute cerebellar degeneration and limbic encephalitis being the most common;19(40.4%)patients showed non-classical syndrome,with sensorimotor neuropathy being the most common.For patients with classical syndrome,the central nervous system was mainly involved,while for patients with non-classical syndrome,the peripheral nervous system was often involved.Neuroparatumor antibodies were detected in 40 of the 47 patients,of which 28 were positive for characteristic antibodies.The detection rate of anti-Hu antibody(21%)was the highest,followed by anti-Yo antibody(14.9%).A total of 12 cases were positive for other non-characteristic antibodies,and 3 cases were positive for double antibodies.Of the 47 patients,41 received tumor therapy and/or immunotherapy,of which 31(31/41;75.6%)had their clinical symptoms relieved one month after treatment,and the differences in mRS scores before and after treatment that were tested by Wilcoxon rank test were statistically significant(P<0.05).Of the 47 patients,31 had good short-term prognosis(31/47;66.0%)and 16 had poor short-term prognosis(16/47;34.0%).The clinical data of the two groups of patients with good and poor short-term prognosis were compared and analyzed,and there was a significant difference in whether the two subgroups of patients received tumor and/or treatment(P<0.05).Conclusion PNS is commonly found in male patients,and mostly occurs in middle-aged and elderly patients,who have subacute and chronic oneset.Most complicated tumors are lung cancers,of which the clinical manifestations are complex and diverse.The detection of neural paraneoplastic antibodies is vital to the diagnosis of PNS.For patients diagnosed with PNS,tumor therapy and/or immunotherapy should be carried out as soon as possible to relieve the clinical symptoms and improve the prognosis of the patients.