以球麻痹起病的副肿瘤综合征  

Paraneoplastic neurologic syndromes with bulbar palsy as the presenting symptom

作　　者：蔡林君 陈虹西 石紫燕 杜琴 周红雨[1] CAI Linjun;CHEN Hongxi;SHI Ziyan;DU Qin;ZHOU Hongyu(Department of Neurology,West China Hospital,Sichuan University,Chengdu Sichuan 610041,China)

机构地区：[1]四川大学华西医院神经内科,610041

出　　处：《中国神经免疫学和神经病学杂志》2020年第2期109-112,共4页Chinese Journal of Neuroimmunology and Neurology

基　　金：国家重点研发计划(2017YFC0907704);四川省科技厅-重点项目(2018SZ0388)。

摘　　要：目的归纳总结以球麻痹起病的神经系统副肿瘤综合征(PNS)的临床特点及转归,为临床进一步认识PNS提供依据。方法回顾分析作者医院2010-2019年收治的以球麻痹起病的符合PNS诊断标准的患者,按照是否符合重症肌无力(MG)诊断标准,分为MG组及非MG组;比较两组间临床特征、实验室检查指标、治疗、预后的差别。结果收集随访PNS患者20例,其中MG组患者11例,平均发病年龄(39.0±14.0)岁,以女性为主〔8例(72.7%)〕;非MG组共9例,平均发病年龄为(60.0±15.0)岁,以男性为主〔8例(88.9%)〕;非MG组肿瘤标志物阳性率高于非MG组(18.1%比77.8%,P=0.031)。MG组均接受了外科干预治疗;两组中均有部分患者使用激素及(或)丙种球蛋白治疗,痊愈及好转患者比例差异无统计学意义(P>0.05)。MG组无死亡,非MG组死亡2例。结论在以球麻痹首发的PNS患者中,符合MG的患者女性多见,外科手术联合免疫抑制剂能显著改善患者预后,而不符合MG者以老年男性多见,肿瘤标志物阳性率更高,预后及死亡率更差。Objective To summarize the clinical characteristics and prognosis of paraneoplastic neurologic syndromes(PNS)causing bulbar palsy,so as to provide evidence for further clinical understanding of PNS.Methods The patients with bulbar palsy who met the PNS diagnostic criteria in our hospital from 2010 to 2019 were divided into a myasthenia gravis(MG)group and non-MG group according to whether they met the diagnostic criteria of MG or not,and the differences of clinical features,laboratory examination indexes,treatment and prognosis between the two groups were compared.Results There were 20 patients with PNS,including 11 patients in the MG group,the average age of onset in the MG group was(39.0±14.0)years,mainly female[8 cases(72.7%)],all of the MG patients received surgical intervention,9 cases weve in the non-MG group,the average age of onset was(60.0±15.0)years,mainly male[8 cases(88.9%)].The non-MG group had a higher positive rate of tumor markers(18.1%vs.77.8%P=0.031),worse prognosis and higher mortality.Conclusions In PNS patients presenting with bulbar palsy,most of the MG patients are women,surgery combined with immunosuppressant therapy is the first choice for treatment;non-MG is more common in elderly men,the positive rate of tumor markers is higher,the prognosis and mortality are worse.

关 键 词：神经系统副肿瘤综合征 球麻痹 重症肌无力 治疗 预后 

分 类 号：R741[医药卫生—神经病学与精神病学]