文章摘要
赵鑫 ，刘力玮 ，朱舟 ，朱遂强.抗CV2自身免疫性脑炎合并重症肌无力一例并文献复习[J].神经损伤功能重建,2020,15(11):621-626
抗CV2自身免疫性脑炎合并重症肌无力一例并文献复习
Anti-CV2 Antibody-Associated Autoimmune Encephalitis with Myasthenia Gravis: a CaseReport and Literature Review
  
DOI：
中文关键词: 自身免疫性脑炎  重症肌无力  CV2抗体
英文关键词: autoimmune encephalitis  myasthenia gravis  CV2 antibody
基金项目:国家重点研发项 目（No. 2017YFC1 310000）； 国家自然科学基 金（No. 82001218）
作者单位
赵鑫a ，刘力玮b ，朱舟a* ，朱遂强a* 华中科技大学同 济医学院附属同 济医院 a. 神经内 科，b.病理科 
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中文摘要:
      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。
英文摘要:
      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.
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