应用副肿瘤综合征更新标准分析抗CV2/CRMP5抗体相关副肿瘤综合征的临床异质性

Clinical heterogeneity of anti-CV2/CRMP5 antibody associated paraneoplastic syndrome

目的应用2021年副肿瘤综合征诊断标准分析抗CV2/CRMP5抗体相关副肿瘤综合征的临床表现、影像学和血清学特征。方法收集作者医院2020年6月至2021年1月收治的抗CV2/CRMP5抗体相关副肿瘤综合征患者5例,分析其临床表现、血清学和影像学资料特征。采用2021年更新副肿瘤综合征诊断标准及评分(PNS-Care Score)进行验证。结果5例患者中,其中男1例,女4例,年龄42~59岁。临床表现为快速进展性痴呆伴低热1例,视神经脑脊髓病1例,视神经脊髓病1例,重症肌无力合并胸腺瘤AB型1例,视神经病合并小细胞肺癌1例。3例未发现明确肿瘤。除1例重症肌无力合并胸腺瘤外,余4例患者均行腰穿脑脊液(CSF)检查,其中CSF白细胞升高1例,蛋白升高2例(600~676 mg/L)。1例血清Amphiphysin弱阳性,1例血清抗Hu抗体弱阳性,1例血清和CSF抗CV2/CRMP5抗体均阳性。表现为重症肌无力合并胸腺瘤的患者抗乙酰胆碱受体抗体和抗Titin抗体阳性。5例患者PNS-Care评分8~11分,全部达到确诊副肿瘤综合征标准。结论抗CV2/CRMP5抗体相关神经系统副肿瘤综合征患者存在高度临床异质性,部分患者伴发肿瘤。应加强对本病的认识和长期随访。副肿瘤PNS-Care评分有助于临床诊断。

Objective To study the clinical,imaging and serological features of anti-CV2/CRMP5 paraneoplastic syndrome.Methods We collected the clinical data of the inpatients with anti-CV2/CRMP5 paraneoplastic syndrome in Beijing Tongren Hospital from June 2020 to February 2021,and systematically analyzed their clinical manifestations,serological and imaging data.Results There were 1 male and 4 females,aged 42-59 years.There were 1 case of rapid progressive dementia with low fever,1 case of optic nerve involvement and encephalomyelitis,1 case of optic neuromyelitis,1 case of myasthenia gravis combined with thymoma type AB,1 case of optic neuritis combined with small cell lung cancer.One case of small cell lung cancer and one case of AB thymoma were found respectively,and no definite tumor was found in the other 3 cases.Except for the case of myasthenia gravis complicated with thymoma,lumbar puncture was performed in the other 4 patients,including 1 case of cerebrospinal fluid leukocyte elevation and 2 cases of cerebrospinal fluid protein elevation(600-676 mg/L).One case was weakly positive for serum amphiphysin antibody,one case was weakly positive for anti-Hu antibody,and one case was positive for serum and cerebrospinal fluid anti-CV2/CRMP5 antibody.The patient with thymoma and myasthenia gravis was positive for anti-acetylcholine receptor antibody and anti-titin antibody.Conclusions PNS of anti-CV2/CRMP5 antibody has high clinical heterogeneity,and some patients have tumor.This disease is rarely reported in China,so we should strengthen the understanding of this disease and long-term follow-up.