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
高晓宇,郑雪姣,狄政莉,薛秀云,刘志勤
摘要(Abstract):
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。
关键词(KeyWords): 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
基金项目(Foundation):
作者(Author): 高晓宇,郑雪姣,狄政莉,薛秀云,刘志勤
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