PT -期刊文章盟大卫Chaar AU - Mihir Kakara盟Shitiz Sriwastava AU - Sasan Moshirzadeh AU -米甲哈龙盟罗伯特Lisak TI - Anti-MAG相关神经病变患者的特征响应利妥昔单抗(5242)DP - 2020年4月14日TA -神经病学PG - 5242 VI - 94 IP - 15补充4099 - //www.ez-admanager.com/content/94/15_Supplement/5242.short 4100 - http://首页www.ez-admanager.com/content/94/15_Supplement/5242.full所以Neurology2020 4月14日;94 AB -目的:评估患者的长期反应与利妥昔单抗和描述的特征与有利的结果。背景:Anti-myelin相关糖蛋白(MAG)神经病变是一种使人衰弱的paraproteinemic脱髓鞘病变。免疫疗法的效果一直不一致的多个研究;然而,rituximab-a b细胞消耗经纪人被证明是有益的在病人的一个子集。设计/方法:7 anti-MAG神经病变患者利妥昔单抗的病例分析。记录了人口、血清学和电诊法的特性。结果来评估疗效包括改良Rankin规模(夫人)和炎性病变病因和治疗(INCAT)规模。结果:86%的患者都是白人男性。在诊断上,平均年龄57岁,平均anti-MAG效价在急救员和1:513,600 1:40,000无(p = 0.04)。疾病治疗开始前4±3年的反应,相比11±7年无(p = 0.05)。滴度下降了7.5±4.7折后治疗。在12个月的治疗后,病人4/7报道症状改善感觉异常、异常性疼痛、震颤和步态。 Neurologic examination demonstrated recovery of distal strength, vibration and proprioception with resolution of Romberg’s sign, and re-emergence of reflexes in the lower extremities. INCAT (mRS) score improved by 1.8±1.0, (p=0.01) (1.3±0.5, p=0.004) points from a baseline of 3.0±1.6 (2.3±0.5). Terminal latency index (TLI) of the median nerve was 0.30±0.05. The remaining patients did not have further deterioration in their symptoms, exam or scores. One patient had lambda light-chains, unlike the others with kappa light-chains, and did not improve. Another elderly patient with TLI of 0.18 did not respond either despite a 9-fold reduction in anti-MAG titer.Conclusions: Features of rituximab responders include shorter disease-duration, lower anti-MAG titers and TLI>0.25. Non-responders did not deteriorate further and remained stable. This retrospective case-series supports rituximab as a viable option in a subset of patients for management of anti-MAG neuropathy, with improvements in symptoms, neurological exam and functional neuropathy scales.Disclosure: Dr. Chaar has nothing to disclose. Dr. Kakara has nothing to disclose. Dr. Sriwastava has nothing to disclose. Dr. Moshirzadeh has nothing to disclose. Dr. Halon has nothing to disclose. Dr. Lisak has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion, Argenix, Novartis, Syntimmune, Teva Pharmaceuticals (non-branded talks), Alpha Sites, Clearview Consulting, Destum, GLG consulting, Haven Consulting, Informa Consulting, Insights Pharma Consulting, Slingshot Consulting. Dr. Lisak has received research support from Catalyst, Chungai, Genentec/Roche, Mallinckdrot, Medimmune, Novartis, Ra Pharmaceuticals, Teva Phamaceuticals.