抗SOX-1,GABABR和VGCC抗体阳性的副肿瘤综合征:病例报告与文献回顾

Paraneoplastic neurological syndrome with positive SOX-1,GABABR,and VGCC antibodies:A case report and literature review

目的神经系统副肿瘤综合征是罕见的由潜在肿瘤诱发的免疫介导的疾病。早期识别特殊的副肿瘤综合征亚型和特异性的自身抗体可以指导高效的肿瘤筛查,进而促进及时的抗肿瘤治疗并延长生存期。方法回顾潍坊市人民医院神经内科收治的1例合并多个临床表型及多种抗体的副肿瘤综合征患者的诊治过程,同时对相关文献进行系统性综述。结果患者以行走不稳和精神行为异常为早期突出表现,最初诊断为伴抗SOX-1及GABABR抗体阳性的副肿瘤性小脑变性和副肿瘤性边缘性脑炎,因电生理检查的异常发现和P/Q型VGCC抗体阳性,考虑合并存在Lambert-Eaton肌无力综合征,所有证据均指向该患者存在隐匿的小细胞肺癌。最终胸腔镜活检证实PET-CT上显示的肿大淋巴结符合小细胞肺癌的病理改变。该患者同时接受了抗肿瘤治疗和免疫治疗,生存期为19个月。结论检测发现抗SOX1抗体阳性应强化对SCLC的筛查。细胞表面抗体介导的副肿瘤性边缘性脑炎和副肿瘤性神经肌肉接头病变患者在抗肿瘤治疗的同时应立即启动免疫治疗,预后相对较好。

Objective Paraneoplastic neurological syndrome is a rare immune-mediated disease induced by underlying tumors,and early identification of special subtypes of paraneoplastic syndrome and specific autoantibodies can guide efficient tumor screening,thereby promoting timely antitumor treatment and prolonging survival time.Methods A retrospective analysis was performed for the diagnosis and treatment of a case of paraneoplastic neurological syndrome with multiple clinical phenotypes and multiple autoantibodies who was admittd to Department of Neurology,Weifang People’s Hospital,and a systmatical review was performed for related articles.Results The patient presented with unsteady gait and mental and behavioral disorders in the early stage and was initially diagnosed with paraneoplastic cerebellar degeneration and paraneoplastic limbic encephalitis with positive anti-SOX-1 and anti-GABABR antibodies.Due to abnormal electrophysiological results and positive P/Q-type VGCC antibodies,the patient was considered to have Lambert-Eaton myasthenic syndrome,and all evidence suggested that the patient had occult small cell lung cancer.Finally thoracoscopic biopsy confirmed that lymph node enlargement on PET-CT was in accordance with the pathological changes of small cell lung cancer.The patient received both antitumor treatment and immunotherapy and had a survival time of 19 months.Conclusion The detection of anti-SOX1 antibodies should promote the screening for small cell lung cancer.In patients with paraneoplastic limbic encephalitis and paraneoplastic neuromuscular syndromes mediated by cell surface antibodies,immunotherapy should be initiated in parallel with antitumor treatment,and such patients tend to have a good prognosis.