抗CV2自身免疫性脑炎合并重症肌无力一例并文献复习

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。

Anti-CV2 Antibody-Associated Autoimmune Encephalitis with Myasthenia Gravis: a CaseReport and Literature Review

To analyze the clinical characteristics and prognosis of anti-CV2 antibody-associatedparaneoplastic neurological syndrome (PNS). Methods: One case of anti-CV2 antibody-associated PNS wasreported and the relevant literatures were reviewed. Results: The patient of the case study presented withcerebellar ataxia and autoimmune encephalitis at her first visit. Serum anti-CV2 antibody was positive, and athymus mass was detected by whole-body PET-CT. Pathological biopsy revealed this to be a thymic cyst. Thepatient showed marked improvement following thymectomy. Two years later she was admitted for generalizedfatigue and showed positive serum anti-CV2, anti-AChR, and anti-Titin antibodies. The patient was diagnosedwith myasthenia gravis, and her condition improved significantly after immunotherapy. Literature review found92 reported cases of anti-CV2 antibody-associated PNS. Common clinical features included peripheralneuropathy, chorea, cerebellar ataxia, autoimmune encephalitis, optic neuritis or retinal symptoms, andmyasthenia 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 withmuscle weakness, limbic encephalitis, and cerebellar ataxia. Recurring PNS is very rare, and barring tumorrelapse, immunotherapy may improve its prognosis.