摘要
目的:探讨CV2抗体相关神经系统副肿瘤综合征(PNS)的临床特征及预后特点。方法:本文报道一例CV2抗体相关PNS患者,结合文献进行回顾性分析。结果:本文患者首诊时表现为小脑性共济失调和边缘性脑炎的症状,CV2抗体阳性,全身PET-CT提示胸腺占位,病理活检示胸腺囊肿,胸腺切除术后症状迅速好转。但2年后患者出现全身无力,CV2抗体、AChR抗体及Titin抗体阳性,诊断为重症肌无力,经免疫治疗后明显好转。既往报道的CV2抗体相关神经系统副肿瘤综合征患者共92例,常见的临床症状包括周围神经病、舞蹈症、小脑性共济失调、自身免疫性脑炎、视神经炎或视网膜病变和肌无力综合征,常见的肿瘤类型包括小细胞肺癌和胸腺瘤。结论:CV2抗体相关PNS临床表现多样,伴胸腺占位时主要表现为肌无力、边缘性脑炎和小脑性共济失调。复发性PNS十分罕见,当排除肿瘤复发时给予免疫治疗,预后相对较好。
Objective:To analyze the clinical characteristics and prognosis of anti-CV2 antibody-associated paraneoplastic neurological syndrome(PNS).Methods:One case of anti-CV2 antibody-associated PNS was reported and the relevant literatures were reviewed.Results:The patient of the case study presented with cerebellar ataxia and autoimmune encephalitis at her first visit.Serum anti-CV2 antibody was positive,and a thymus mass was detected by whole-body PET-CT.Pathological biopsy revealed this to be a thymic cyst.The patient showed marked improvement following thymectomy.Two years later she was admitted for generalized fatigue and showed positive serum anti-CV2,anti-AChR,and anti-Titin antibodies.The patient was diagnosed with myasthenia gravis,and her condition improved significantly after immunotherapy.Literature review found 92 reported cases of anti-CV2 antibody-associated PNS.Common clinical features included peripheral neuropathy,chorea,cerebellar ataxia,autoimmune encephalitis,optic neuritis or retinal symptoms,and myasthenia syndrome.Common tumor types included small-cell lung cancer and thymoma.Conclusion:Anti-CV2 antibody-associated PNS exhibits various clinical symptoms;thymoma is often associated with muscle weakness,limbic encephalitis,and cerebellar ataxia.Recurring PNS is very rare,and barring tumor relapse,immunotherapy may improve its prognosis.
作者
赵鑫
刘力玮
朱舟
朱遂强
ZHAO Xin;LIU Li-wei;ZHU Zhou;ZHU Sui-qiang(Department of Neurology,Tongji Hospital,Tongji Medical College,Huazhong University of Science&Technology,Wuhan 430030,China;Department of Pathology,Tongji Hospital,Tongji Medical College,Huazhong University of Science&Technology,Wuhan 430030,China)
出处
《神经损伤与功能重建》
2020年第11期621-626,共6页
Neural Injury and Functional Reconstruction
基金
国家重点研发项目(No.2017YFC1310000)
国家自然科学基金(No.82001218)。