抗HU抗体阳性副肿瘤性小脑变性1例及文献复习  

One case of anti-HU antibody positive paraneoplastic cerebellar degeneration and literature review

作　　者：高晓宇 郑雪姣 狄政莉[1] 薛秀云 刘志勤[1] GAO Xiaoyu;ZHENG Xuejiao;DI Zhengli;XUE Xiuyun;LIU Zhiqin(Xi’an Central Hospital,Xi’an 710003,China)

机构地区：[1]西安市中心医院

出　　处：《中国实用神经疾病杂志》2019年第10期1148-1151,共4页Chinese Journal of Practical Nervous Diseases

摘　　要：副肿瘤性小脑变性(paraneoplastic cerebellar degenertion,PCD)又称亚急性小脑变性(subacute cerebellar degeneration,SCD),是神经系统副肿瘤综合征(praneoplastic neurological syndrome,PNS)引起神经系统的典型损害之一,神经系统副肿瘤综合征(PNS)是一种罕见的副肿瘤综合征,并不是由肿瘤转移或直接浸润到神经系统引起,是由免疫反应的改变引起。是一种与恶性肿瘤相关但原因不明的小脑非转移性病变。本文报道1例以副肿瘤性小脑变性为首发症状的肺癌病例,加强临床医生对该病的认识。该患者即以双下肢行走不稳收入院,表现为小脑受损的症状与体征,脑干受损症状与体征未表现。检查脑脊液生化示,葡萄糖4.73 mmol/L,微量总蛋白548.00 mg/L;脑脊液抗酸、墨汁染色阴性。脑脊液常规示,红细胞数0,白细胞26个/mm^3;副肿瘤相关抗体抗Hu抗体阳性。行胸部增强CT示,左肺上叶前段占位病变,考虑新生物;左肺上叶尖后段纤维条灶索;纵隔内及左肺门区多发淋巴结肿大。结合患者临床表现和辅助检查,临床诊断为副肿瘤性小脑变性。早期诊断副肿瘤性小脑变性对于及时发现和根除宿主体内的肿瘤至关重要,尤其小细胞肺癌、乳腺癌、卵巢癌等。有条件可在血液和脑脊液中检测特定的抗神经元抗体,目前发现许多抗神经元抗体与PCD有关,并且对特定的肿瘤类型的早期诊断提供依据,如抗Hu抗体提示小细胞肺癌,抗Yo抗体提示乳腺、卵巢等肿瘤。该病总体预后不良,应积极采取综合治疗手段,控制肿瘤的转移及复发,以提高患者生存质量,延长生存时间,改善生存率。Paraneoplastic cerebellar degeneration (PCD),also known as subacute cerebellar degeneration (SCD),is one of the typical neurological lesions caused by praneoplastic neurological syndrome (PNS).It is not caused by tumor metastasis or direct infiltration into the nervous system,but by changes in the immune response.It is a nonmetastatic cerebellar lesion associated with malignancy but of unknown cause.This paper reported a case of lung cancer with para neoplastic cerebellar degeneration as the first symptom,which strengthened clinicians’ understanding of the disease.The patient was admitted to the hospital with unstable walking of both lower limbs,showing symptoms and signs of cerebellar damage,but no signs and symptoms of brain stem damage.Cerebrospinal fluid biochemical examination showed that glucose was 4.73 mmol/L and total trace protein was 548.00 mg/L.Cerebrospinal fluid anti acid,ink stain negative.Routine cerebrospinal fluid showed a red blood cell count of 0 and a white blood cell count of 26 /mm 3.The anticancer antibody against Hu was positive.Chest enhanced CT was performed,and the lesions in the anterior segment of the upper lobe of the left lung were occupied,and new organisms were considered (figure 1).Fibrous bands of the posterior segment of the upper lobe of the left lung;Multiple lymph node enlargement in the mediastinum and hilum of the left lung.Combined with clinical manifestations and auxiliary examinations,the patient was clinically diagnosed as paraneoplastic cerebellar degeneration.Early diagnosis of para neoplastic cerebellar degeneration is crucial for the timely detection and eradication of tumors in the host,especially small cell lung cancer,breast cancer,ovarian cancer,etc.It is possible to detect specific anti neuronal antibodies in blood and cerebrospinal fluid under certain conditions.At present,many anti neuronal antibodies are found to be related to PCD,and provide basis for the early diagnosis of specific tumor types,such as anti hu antibody indicating small cell lung cancer,

关 键 词：副肿瘤性小脑变性 亚急性小脑变性 抗HU抗体 副肿瘤综合征 肺癌 

分 类 号：R742[医药卫生—神经病学与精神病学]