经典与非经典副肿瘤神经综合征临床特点分析

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目的:探讨经典与非经典副肿瘤神经综合征(PNS)的临床特征。方法:回顾性分析2015年至2020年中国科学技术大学附属第一医院收治的48例确诊PNS,总结其临床特点,进行关于分型、临床表现、神经副肿瘤抗体、原发肿瘤等的系统性分析。并按照Graus标准将纳入病例分为经典和非经典PNS组,比较两组之间临床特点、抗体阳性率、肿瘤合并率、确诊时间、死亡率等差异。结果:48例PNS中,21例(43.8%)血清特征性抗体阳性,其中经典PNS 28例(58.3%),非经典PNS 20例(41.7%)。两组间在年龄、性别、临床累及部位、特征性阳性抗体类型、肿瘤确诊率及随访死亡率上差异均无统计学意义(n P>0.05)。非经典PNS组确诊时间为[n M(n Q1, Q3)] 3.0 (2.0, 6.5)个月,明显长于经典PNS组1.0(0.6, 3.0)个月(n P0.05). The time of diagnosis in the non-classical PNS group was 3.0 (2.0, 6.5) months, which was significantly longer than that in the classical PNS group 1.0(0.6, 3.0) months (n P<0.05). Meanwhile, the combination rate of non-characteristic antibodies in the classical PNS group (10 cases, 35.7%) was significantly higher than that in the non-classical PNS group (1 case, 5.0%) (n P=0.016). During the follow-up, 39 patients (81.3%) with tumor were confirmed, and 29 patients (60.4%) were diagnosed with PNS before the tumor was found.n Conclusions:The“non-classical”PNSs are common in clinical settings. Diagnosis may be delayed due to the nonclassical symptoms of the patients. When patients have clinical symptoms related to PNS, onconeural antibodies should be detected and the relevant tumors should also be screened. Patients have positive antibodies but with no tumors should be closely followed up for more than 5 years.
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