神经系统副肿瘤综合征与神经副肿瘤抗体分析

The clinical analysis of paraneoplastic neurological syndromes and onconeuronal antibodies

目的探讨神经系统副肿瘤综合征(PNS)临床表现及神经副肿瘤抗体的特征。方法分析2009年1月至2019年9月北京大学第一医院神经内科确诊的110例PNS患者的临床特征及神经副肿瘤抗体类型。结果110例患者出现17种临床综合征,其中43例(39.1%)为经典临床综合征,最常见的为亚急性小脑变性14例(12.7%)和边缘性脑炎11例(10.0%);62例(56.4%)为非经典临床综合征,最常见的为感觉运动周围神经病19例(17.3%)和重症肌无力10例(9.1%)。69例(62.7%)患者发现恶性肿瘤,肺癌20例(18.2%)及血液系统肿瘤12例(10.9%)为最常见的肿瘤。71例(64.5%)患者在血液中检测到特征性副肿瘤抗体,最常见的为抗Amphiphysin抗体26例(23.6%)和抗Yo抗体24例(21.8%)。结论临床表现为非经典副肿瘤综合征的患者,亦应积极进行特征性副肿瘤抗体的检测,并根据抗体结果对特定肿瘤进行重点筛查。

Objective To investigate the clinical manifestations of paraneoplastic neurological syndromes(PNS)and the characteristics of onconeuronal antibodies.Methods 110 patients in Peking University First Hospital were diagnosed as PNS from January 2009 to September 2019;their clinical features and characteristics of onconeuronal antibodies were analyzed.Results 17 different kinds of clinical syndromes were found in 110 patients,of which 43(39.1%)were classical syndromes.The most common classical syndrome were subacute cerebellar degeneration(12.7%)and limbic encephalitis(10.0%).Non-classical clinical syndromes were found in 62(56.4%)patients,and the most common of which were sensory motor peripheral neuropathy(17.3%)and myasthenia gravis(9.1%).Sixty nine(62.7%)patients were found with malignant tumor,the most common of which were lung cancer(18.2%)and hematological system tumors(10.9%).71 patients(64.5%)had well-characterized onconeuronal antibodies in their serum,the most common of which were anti-Amphiphysin(23.6%)and anti-Yo antibodies(21.8%).Conclusions Even in patients with non-classical paraneoplastic syndrome,we should also detect onconeuronal antibodies and screen for the specific tumors according to the antibody results.