副肿瘤性神经综合征60例临床回顾

The clinical analysis of 60 cases of paraneoplastic neurological syndrome

目的 探讨副肿瘤性神经综合征（PNS）的临床特征.方法 采用国际统一PNS的Graus诊断标准,回顾性分析2008年1月至2012年12月北京协和医院神经内科收治的60例PNS患者的临床特征.结果 60例PNS患者出现11种常见的副肿瘤临床综合征,其中29/60例（48.3％）表现为经典临床综合征,最常见的为边缘叶脑炎（11/60,18.3％）,其次为亚急性小脑变性（10/60,16.7％）;27/60例（45.0％）表现为非经典临床综合征,最常见的为亚急性/慢性感觉运动性周围神经病13/60（21.7％）.60例PNS患者中33例（55.0％）明确原发肿瘤,其中肺癌占39.4％（13/33）,为最常见的肿瘤类型,而肺癌中小细胞肺癌占6/13.入组的60例患者中,19例（31.7％）在血液与脑脊液中检测到特征性抗体,以抗Hu、Yo抗体阳性率最高（16/19）;无患者检测到非特征性抗体;8/60例（13.3％）检测到神经元表面抗体,均为抗N-甲基-D-天冬氨酸受体抗体.31例患者随访至今,因PNS相关死亡者共11例（35.5％）,其中“确诊的PNS”患者7例死亡（7/15）,“可能的PNS”患者4例死亡（4/16）,差异无统计学意义（χ^2=0.782,P=0.380）.患者的1年累积生存率为84.0％,3年累积生存率为61.0％,5年累积生存率为54.0％.结论 无论PNS患者临床上表现为经典或非经典副肿瘤临床综合征,均应进行肿瘤神经抗体的检测,并进行系统的肿瘤筛查,或者根据抗体检测结果对特定肿瘤进行重点筛查.对于未能找到原发肿瘤的PNS患者,应进行密切随访.

Objective To explore the clinical features of paraneoplastic neurological syndrome （PNS）.Methods Sixty PNS patients in Peking Union Medical College Hospital from January 2008 to December 2012 according to the international diagnostic criteria for PNS recommended by Graus were diagnosed and their clinical features were retrospectively analyzed.Results Sixty PNS patients had 11 different clinical syndromes,including 29/60 （48.3%） classical syndromes and 27/60 （45.0%） nonclassical syndromes.The most common classical syndromes were limbic encephalitis （11/60,18.3%） and subacute cerebellar degeneration （10/60,16.7%）,while the most common non-classical syndrome was subacute/chronic sensorimotor neuropathies.Thirty-three of sixty （55.0%） PNS patients were found with underlying tumors,the most common of which was lung cancer （13/33,39.4%）,and 46.2% （6/13） of lung cancer was small cell lung cancer.And 19/60 （31.7%） PNS patients had well characterized onconeuronal antibodies in their serum/cerebral spinal fluid,and anti-Hu and anti-Yo antibodies were most commonly seen （16/19）.No partially characterized onconeuronal antibody was found.Eight of sixty （13.3%） patients were found with neuronal cell-surface antibodies,all of which were anti-N-methyl-Daspartate receptor antibodies.Eleven of thirty-one followed-up patients died,including 7 （7/15） definite PNS and 4 （4/16） possible PNS,without significant difference between groups （χ^2 =0.782 P =0.380）.The 1-,3-and 5-year survival rates were 84.0%,61.0% and 54.0% respectively.Conclusions PNS patients with either classical or non-classical syndromes should be screened for onconeural antibodies and underlying tumors.Those patients without underlying tumor should be closely followed up.