合并皮肤色素沉着的抗Hu、抗Amphiphysin抗体阳性副肿瘤性感觉神经元病一例  

Case report:anti-hu,anti-amphiphysin antibodies related paraneoplastic sensory neuronopathy combined with skin pigmentation

作　　者：徐宁康 兰希福[2] 赵曼丽[2] 徐谦[3] 孙翀 Xu Ninghkang;Lan Xifu;Zhao Manli;Xu Qian;Sun Chong(First Clnical Medical College,Shandong University of Traditional Chinese Medicine,Jinan 250355,China)

机构地区：[1]山东中医药大学第一临床医学院,济南250300 [2]山东潍坊市中医院脑病科 [3]山东潍坊市人民医院肿瘤内科 [4]上海复旦大学附属华山医院神经内科

出　　处：《脑与神经疾病杂志》2024年第9期547-551,共5页Journal of Brain and Nervous Diseases

基　　金：全国中医临床优秀人才研修项目(国中医药人教函[2022]1号);山东省中医药科技发展计划项目(2019-0706)。

摘　　要：目的 提高对合并罕见临床症状的双抗体阳性副肿瘤感觉神经元病(PSN)的认识。方法 报道1例合并皮肤色素沉着的双抗体阳性PSN患者,分析其临床资料。结果 患者女性,58岁,以四肢不对称性麻木无力为主要表现,查血清副肿瘤抗体谱:抗Hu抗体(+)、抗Amphiphysin抗体(+),查体发现患者双手及腹部皮肤色素沉着,结合病史及检查结果高度怀疑副肿瘤综合征,但初步检查未发现肿瘤病灶,应用地塞米松及免疫球蛋白后效果不佳,行血浆置换后症状减轻。6个月后患者复查PET-CT发现存在肺部肿瘤,纵隔淋巴结活检病理证实为小细胞肺癌,诊断为“PSN”,后患者转入肿瘤科进一步治疗。结论 高风险副肿瘤自身抗体如抗Hu、抗Amphiphysin抗体对副肿瘤综合征的诊断具有重要意义,不明原因的皮肤色素沉着应引起临床医生的重视,警惕副肿瘤综合征的可能。Objective To improve the understanding of double-antibody-positive paraneoplastic sensory neuronopathy(PSN)with rare clinical symptoms.Methods A case of double-antibody-positive PSN in a patient with skin pigmentation was reported,and its clinical data were analyzed.Results The patient was a 58-year-old female patient with asymmetric numbness and weakness of the limbs as the main manifestation.The serum paraneoplastic antibody spectrum was checked:anti-Hu antibody(+),anti-amphiphysin antibody(+).Physical examination revealed pigmentation on the skin of the patient's hands and abdomen.Combining the medical history and examination results,paraneoplastic syndrome was highly suspected.However,no tumor lesions were found in the initial examination,and the treatment with dexamethasone and immunoglobulin had poor results.The patient's symptoms were relieved after plasma exchange.Six months later,the patient's PET-CT reexamination revealed the presence of lung tumors,and mediastinal lymph node biopsy pathology confirmed small cell lung cancer.The patient was diagnosed as"PSN",and the patient was later transferred to the oncology department for further treatment.Conclusion High-risk paraneoplastic autoantibodies,such as anti-hu and anti-amphiphysin antibodies,play a crucial role in the diagnosis of paraneoplastic syndrome.Clinicians should be vigilant about unexplained skin pigmentation,as it may be indicative of underlying paraneoplastic syndrome.

关 键 词：副肿瘤神经系统综合征 副肿瘤自身抗体 皮肤色素沉着 

分 类 号：R745.7[医药卫生—神经病学与精神病学]