Anti-CD20 monoclonal antibody (rituximab) is effectively used in the treatment of B-cell lymphomas. Recent reports in the literature suggest that antibody associated autoimmune disorders may respond to rituximab. We t...Anti-CD20 monoclonal antibody (rituximab) is effectively used in the treatment of B-cell lymphomas. Recent reports in the literature suggest that antibody associated autoimmune disorders may respond to rituximab. We therefore treated nine patients with anti-Hu or anti-Yo associated paraneoplastic neurological syndromes (PNS) with a maximum of four monthly IV infusions of rituximab (375 mg/m2). In this uncontrolled, unblinded trial of rituximab, three patients improved ≥ 1 point on the Rankin Scale (RS). One patient with limbic encephalitis improved dramatically (RS from 5 to 1). Further studies of rituximab in autoantibody associated PNS are warranted.展开更多
神经系统副肿瘤综合征（paraneoplastic neurologicalsyndrome,PNS）是肿瘤通过远隔效应引起的神经系统并发症,既无局部瘤细胞转移压迫浸润,也无代谢、感染或治疗相关损伤等其他病因.20世纪80年代以来,以Hu抗体为代表的一批肿瘤神经元...神经系统副肿瘤综合征（paraneoplastic neurologicalsyndrome,PNS）是肿瘤通过远隔效应引起的神经系统并发症,既无局部瘤细胞转移压迫浸润,也无代谢、感染或治疗相关损伤等其他病因.20世纪80年代以来,以Hu抗体为代表的一批肿瘤神经元抗体（onconeural antibody）被发现,提示肿瘤与神经元交叉抗原所继发的免疫反应是相当一部分PNS的病因[1].虽然尽早发现肿瘤对PNS治疗和预后均有重要影响,但是临床上由于各种原因导致活体组织检查（活检）病理组织无法获得致使诊断延误的例子不在少数.与此同时,近年来新型PNS相关抗体层出不穷,使得越来越多PNS的潜在神经免疫学机制得以揭示[2].我们报道l例以慢性近端肌无力、腱反射减低及自主神经症状为主要表现患者,电生理检查提示感觉神经、单根运动神经、突触前膜、神经根及自主神经受累.其血清PNS抗体筛查明确Y染色体性别决定区相关高迁移率组盒蛋白1（sex-determining region of Y chromosome-related high mobility group box 1,SOX1）抗体阳性,结合全身PET-CT异常及血清神经特异性烯醇化酶（neuron specific enolase,NSE）升高,考虑伴有小细胞肺癌（small cell lung cancer,SCLC）可能性极大,并最终通过随访确认患者短期内死亡.希望通过本例中SOX1抗体的实际应用,为诊断SCLC相关PNS提供借鉴.展开更多
文摘Anti-CD20 monoclonal antibody (rituximab) is effectively used in the treatment of B-cell lymphomas. Recent reports in the literature suggest that antibody associated autoimmune disorders may respond to rituximab. We therefore treated nine patients with anti-Hu or anti-Yo associated paraneoplastic neurological syndromes (PNS) with a maximum of four monthly IV infusions of rituximab (375 mg/m2). In this uncontrolled, unblinded trial of rituximab, three patients improved ≥ 1 point on the Rankin Scale (RS). One patient with limbic encephalitis improved dramatically (RS from 5 to 1). Further studies of rituximab in autoantibody associated PNS are warranted.
文摘神经系统副肿瘤综合征（paraneoplastic neurologicalsyndrome,PNS）是肿瘤通过远隔效应引起的神经系统并发症,既无局部瘤细胞转移压迫浸润,也无代谢、感染或治疗相关损伤等其他病因.20世纪80年代以来,以Hu抗体为代表的一批肿瘤神经元抗体（onconeural antibody）被发现,提示肿瘤与神经元交叉抗原所继发的免疫反应是相当一部分PNS的病因[1].虽然尽早发现肿瘤对PNS治疗和预后均有重要影响,但是临床上由于各种原因导致活体组织检查（活检）病理组织无法获得致使诊断延误的例子不在少数.与此同时,近年来新型PNS相关抗体层出不穷,使得越来越多PNS的潜在神经免疫学机制得以揭示[2].我们报道l例以慢性近端肌无力、腱反射减低及自主神经症状为主要表现患者,电生理检查提示感觉神经、单根运动神经、突触前膜、神经根及自主神经受累.其血清PNS抗体筛查明确Y染色体性别决定区相关高迁移率组盒蛋白1（sex-determining region of Y chromosome-related high mobility group box 1,SOX1）抗体阳性,结合全身PET-CT异常及血清神经特异性烯醇化酶（neuron specific enolase,NSE）升高,考虑伴有小细胞肺癌（small cell lung cancer,SCLC）可能性极大,并最终通过随访确认患者短期内死亡.希望通过本例中SOX1抗体的实际应用,为诊断SCLC相关PNS提供借鉴.
