17例神经系统副肿瘤综合征临床分析

Clinical analysis of 17 cases of paraneoplastic syndrome of nervous system

目的分析神经系统副肿瘤综合征(paraneoplastic syndrome of nervous system,PNSNS)的临床特点,以提高对该病的早期诊断和治疗效果。方法回顾性分析中国科学技术大学附属第一医院神经内科2016年9月-2019年10月收治的17例PNSNS患者的临床表现、实验室检查结果和治疗效果,并作文献复习。结果 17例患者中14例(82.4%)在肿瘤之前出现神经系统症状,3例(17.6%)在肿瘤之后出现神经系统症状。抗Hu抗体、抗Yo抗体;抗Amphiphysin抗体、抗recoverin抗体、抗Titin抗体、抗Ma2抗体、抗SOX-1抗体、抗Zic4抗体阳性例数分别为4(23.5%)、3(17.6%)、2(11.8%)、2(11.8%)、2(11.8%)、2(11.8%)、2(11.8%)、1(5.9%)例。抗Hu抗体、抗Zic4抗体同时阳性者1例(5.9%)。神经系统病变表现为亚急性小脑变性6例(35.2%);感觉运动性周围神经病3例(17.6%);边缘性脑炎2例(11.8%);Lambert-Eaton综合征2例(11.8%);运动神经元病1例(5.9%);多发性肌炎(5.9%)1例;脑干脑炎1例(5.9%);亚急性感觉神经元病1例(5.9%)。结论多数诊断为神经系统副肿瘤综合征的患者神经系统损害症状早于原发肿瘤临床表现,需要对患者进行肿瘤筛查以及长期随访。激素及丙种球蛋白治疗有效。

Objective To analysis the clinical characteristics of paraneoplastic syndrome of nervous system as to improve the early diagnosis and treatment of the disease. Methods The clinical feature, laboratory results and therapeutic effect of 17 patients with paraneoplastic syndrome of nervous system were retrospectively reviewed. Relevant literatures were reviewed. Results Fourteen(82.4%) cases had paraneoplastic neurological syndrom before the diagnosis of tumor and 3(17.6%) cases after tumor diagnosis. There were 4(23.5%), 3(17.6%), 2(11.8%), 2(11.8%), 2(11.8%), 2(11.8%), 2(11.8%) and 1(5.9%) positive cases of anti-Hu, anti-Yo, anti-Amphiphysin, anti-recoverin, anti-Titin, anti-Ma2, anti-SOX-1 and anti-Zic4 antibodies, respectively. There were 1 positive cases of both anti-Hu and anti-Zic4 antibodies. Neurologic changes were subacute cerebellar degeneration in 6 cases(35.2%), sensorimotor peripheral neuropathy in 3 cases(17.6%), limbic encephalitis in 2 cases(11.8%), Lambert-eaton syndrome in 2 case(11.8%), motor neuron disease in 1 case(5.9%), polymyositis in 1 case(5.9%), brainstem encephalitis in 1 case(5.9%) and subacute sensory neuronopathy in 1 case(5.9%). Conclusion Most of the patients who diagnosed as paraneoplastic syndrome of nervous system have neurological damage symptoms earlier than the primary tumor clinical manifestations, which requires tumor screening and long-term follow-up. The hormone and the gamma globulin treatment is effective.