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21例神经系统副肿瘤综合征临床分析

Clinical analysis of 21 cases of neurological paraneoplastic syndrome
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摘要 目的分析神经系统副肿瘤综合征(PNS)的临床特点以提高对该病的早期诊断和治疗效果。方法回顾性分析惠州市第一人民医院和惠州市中心人民医院神经内科2019年10月—2022年10月收治的21例PNS患者的临床表现、实验室检查结果和治疗效果,并作文献回顾。结果21例患者中出现了10种副肿瘤综合征,其中经典综合征占比28.6%(6/21),最多见的是边缘叶脑炎;20例在血液或脑脊液中发现检测到抗神经元抗体,非特征性抗体阳性率最高(12/20),其中以半定量脑组织切片TBA检测阳性率最高(7/20);有特征性抗体的8例以抗Yo抗体阳性率最高(6/8)。21例患者均随访至2023年3月,8例发现原发肿瘤,其中4例在神经系统病变之后。69.25%(9/13)的患者使用糖皮质激素治疗和(或)丙种球蛋白治疗有效。结论21例PNS患者中以非经典综合征占比较多,经典与非经典副肿瘤综合征均应进行肿瘤筛查,未发现肿瘤者应密切随访。非特征性抗体阳性率最高,提示PNS可能仍有许多相关抗体未明确,临床工作中也应对非特征性抗体阳性予以重视。 Objective To analyze the clinical features of paraneoplastic neurological syndrome(PNS)to improve the early diagnosis and treatment of this disease.Methods The clinical manifestations,laboratory results and treatment effects of 21 patients with PNS admitted to Huizhou First People’s Hospital and Huizhou Central People’s Hospital from October 2019 to October 2022 were retrospectively analyzed,and literature review was performed.Results There were 10 paraneoplastic syndromes in 21 patients,of which classical syndrome accounted for 28.6%(6/21),the most common was limbic lobe encephalitis.Anti-neuronal antibodies were detected in blood or cerebrospinal fluid in 20 cases,with the highest positive rate of non-characteristic antibodies(12/20),among which the positive rate of TBA detection by semi-quantitative brain tissue sections was the highest(7/20);Eight cases with characteristic antibodies had the highest positive rate of anti-Yo antibody(6/8).All 21 patients have been followed up so far,and 8 cases have found primary tumors,4 of which were after neurological lesions.There was 69.25%(9/13)of patients responded to hormone therapy or(and)gamma globulin therapy.Conclusions Non-classical syndrome accounts for more patients with PNS,and both classical and non-classical paraneoplastic syndromes should be screened for tumors,and those who have not found tumors should be closely followed.The positive rate of non-characteristic antibodies is the highest,indicating that there may still be many related antibodies in PNS that are not clear,and the positive of non-characteristic antibodies should also be paid attention to in clinical work.
作者 李才明 李丽英 许倩 沈士媛 吴楚妍 刘以恒 LI Caiming;LI Liying;XU Qian;SHEN Shiyuan;WU Chuyan;LIU Yiheng(Department of Neurology,Huizhou First People’s Hospital,Huizhou 516003,China;Department of Clinical Medicine,the Second Clinical College of Medicine,Guangdong Medical University,Dongguan 523808,China;The First Clinical Medical College of Guangdong Medical University,Zhanjiang 524000,China)
出处 《广州医药》 2024年第10期1198-1204,共7页 Guangzhou Medical Journal
基金 惠州市第一人民医院科研培育与创新基金项目(2020JC001)。
关键词 神经系统副肿瘤综合征 相关神经元抗体 神经系统损害 neurological paraneoplastic syndrome associated neuronal antibodies neurological damage
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