Voltage gated calcium channel(VGCC) antibodies are generally associated with Lambert-Eaton myasthenic syndrome. However the presence of this antibody has been associated with paraneoplastic as well as nonparaneoplasti...Voltage gated calcium channel(VGCC) antibodies are generally associated with Lambert-Eaton myasthenic syndrome. However the presence of this antibody has been associated with paraneoplastic as well as nonparaneoplastic cerebellar degeneration. Most patients with VGCC-antibody-positivity have small cell lung cancer(SCLC). Lambert-Eaton myasthenic syndrome(LEMS)is an autoimmune disease of the presynaptic part of the neuromuscular junction. Its classical clinical triadis proximal muscle weakness, areflexia and autonomic dysfunction. Fifty to sixty percent of LEMS patients have a neoplasia, usually SCLC. The co-occurrence of SCLC and LEMS causes more severe and progressive disease and shorter survival than non-paraneoplastic LEMS. Treatment includes 3,4 diaminopyridine for symptomatic purposes and immunotherapy with prednisolone, azathioprine or intravenous immunoglobulin in patients unresponsive to 3,4 diaminopyridine. Paraneoplastic cerebellar degeneration(PCD) is a syndrome characterized with severe, subacute pancerebellar dysfunction. Serum is positive for VGCC antibody in 41%-44% of patients, usually with the co-occurrence of SCLC. Clinical and electrophysiological features of LEMS are also present in 20%-40% of these patients. Unfortunately, PCD symptoms do not improve with immunotherapy. The role of VGCC antibody in the immunopathogenesis of LEMS is well known whereas its role in PCD is still unclear. All patients presenting with LEMS or PCD must be investigated for SCLC.展开更多
目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例...目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例)和强阳性组(16例),根据CSF抗体情况将患者分为CSF抗体阳性组(22例)和阴性组(87例),根据病程将患者分为病程≤3个月组(76例)和病程>3个月组(42例)。分析各组间年龄以及CSF白细胞计数、总蛋白、IgG指数、24 h IgG鞘内合成率、寡克隆区带(CSF-OCB)和白蛋白商(QAlb)间的差异。结果(1)抗体强阳性组CSF-OCB阳性率和QAlb异常率高于阳性组和弱阳性组(P<0.05)。(2)CSF抗体阳性组CSF白细胞、总蛋白、IgG指数、24 h IgG鞘内合成率和QAlb异常率均高于CSF抗体阴性组(P<0.05)。(3)病程≤3个月组CSF白细胞高于病程>3个月组(P<0.01)。结论副肿瘤特征性抗体阳性患者存在中枢神经系统炎性反应,其发病与体液免疫相关,病程早期CSF白细胞计数增高明显,研究CSF相关指标有助于全面了解患者免疫状况。展开更多
文摘Voltage gated calcium channel(VGCC) antibodies are generally associated with Lambert-Eaton myasthenic syndrome. However the presence of this antibody has been associated with paraneoplastic as well as nonparaneoplastic cerebellar degeneration. Most patients with VGCC-antibody-positivity have small cell lung cancer(SCLC). Lambert-Eaton myasthenic syndrome(LEMS)is an autoimmune disease of the presynaptic part of the neuromuscular junction. Its classical clinical triadis proximal muscle weakness, areflexia and autonomic dysfunction. Fifty to sixty percent of LEMS patients have a neoplasia, usually SCLC. The co-occurrence of SCLC and LEMS causes more severe and progressive disease and shorter survival than non-paraneoplastic LEMS. Treatment includes 3,4 diaminopyridine for symptomatic purposes and immunotherapy with prednisolone, azathioprine or intravenous immunoglobulin in patients unresponsive to 3,4 diaminopyridine. Paraneoplastic cerebellar degeneration(PCD) is a syndrome characterized with severe, subacute pancerebellar dysfunction. Serum is positive for VGCC antibody in 41%-44% of patients, usually with the co-occurrence of SCLC. Clinical and electrophysiological features of LEMS are also present in 20%-40% of these patients. Unfortunately, PCD symptoms do not improve with immunotherapy. The role of VGCC antibody in the immunopathogenesis of LEMS is well known whereas its role in PCD is still unclear. All patients presenting with LEMS or PCD must be investigated for SCLC.
文摘目的探讨副肿瘤特征性抗体阳性患者脑脊液(CSF)特征及其在辅助诊断中的价值。方法回顾性分析北京天坛医院实验诊断中心检测的118例副肿瘤特征性抗体阳性患者的临床资料及实验室数据。根据抗体浓度将患者分为抗体弱阳性(79例)、阳性(23例)和强阳性组(16例),根据CSF抗体情况将患者分为CSF抗体阳性组(22例)和阴性组(87例),根据病程将患者分为病程≤3个月组(76例)和病程>3个月组(42例)。分析各组间年龄以及CSF白细胞计数、总蛋白、IgG指数、24 h IgG鞘内合成率、寡克隆区带(CSF-OCB)和白蛋白商(QAlb)间的差异。结果(1)抗体强阳性组CSF-OCB阳性率和QAlb异常率高于阳性组和弱阳性组(P<0.05)。(2)CSF抗体阳性组CSF白细胞、总蛋白、IgG指数、24 h IgG鞘内合成率和QAlb异常率均高于CSF抗体阴性组(P<0.05)。(3)病程≤3个月组CSF白细胞高于病程>3个月组(P<0.01)。结论副肿瘤特征性抗体阳性患者存在中枢神经系统炎性反应,其发病与体液免疫相关,病程早期CSF白细胞计数增高明显,研究CSF相关指标有助于全面了解患者免疫状况。
