TY -的T1 -自身免疫性CRMP5神经病变表型和结果定义从105例JF -神经学乔-神经病学SP - e103 LP - e110做- 10.1212 / WNL。首页0000000000004803六世- 90 - 2非盟- Divyanshu Dubey AU -万带兰a·列侬盟Avi Gadoth AU -肖恩·j·Pittock盟Eoin p·弗拉纳根AU -约翰·e·史迈林盟-安德鲁·麦肯AU -克里斯托弗·j·克莱因Y1 - 2018/01/09 UR - //www.ez-admanager.com/content/90/2/e103.abstract N2 -目标建立的表型和临床结果collapsin re首页sponse-mediator protein-5 (CRMP5)自身免疫性神经病变相比,anti-neuronal核1型(ANNA1)免疫球蛋白G抗体(免疫球蛋白)神经病变。方法CRMP5-IgG患者和/或ANNA1-IgGs被确定在我们的服务电话测试,并综述了医疗记录。结果一百零五CRMP5-IgG神经病变患者(88%的吸烟者;69%患上癌症,最常见的小细胞肺癌[75%])是识别和51 ANNA1-IgG神经病变的患者相比,27 CRMP5-IgG共存。CRMP5痛苦的轴突多神经根神经病患者(65%),主要是不对称发病(84%),神经病变比癌症诊断的185天(范围60 - 540天)。大多数情况下(79%)有中度到重度的神经性疼痛,都在神经性药物(中位数2,范围1 - 4),阿片类药物的39%。神经活检(n = 2)显示微血管炎症与轴突退化。独自ANNA1相比,CRMP5痛苦的神经病变发生率更高(79%比46%,p = 0.008),非对称多神经根神经病(54%比12%,p & lt;0.001)和炎症性脊髓液(脑脊液蛋白质或有核细胞数升高92% vs 60%, p = 0.022)。小脑性共济失调(21%)、脊髓病(19%),和视神经炎和/或视网膜炎(11%)是常见的神经系统的选择。CRMP5病例有显著的疼痛减少,免疫治疗(p & lt; 0.001). Specifically, high-dose corticosteroid administration was associated with improvement/stabilization in neuropathy impairment scores (p = 0.012) (Class IV). Patients with CRMP5 had better 5-year survival than patients with ANNA1 (67% vs 32%, p = 0.012).Conclusion Painful axonal asymmetric polyradiculoneuropathy is established as the major CRMP5 autoimmune neuropathy presentation and is distinguishable from other paraneoplastic neuropathies, including by ANNA1 autoimmunity. Patients with this phenotype should be prompted for CRMP5-IgG testing to assist in early cancer diagnosis.AMPA=a-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid; ANNA1=anti-neuronal nuclear antibody type 1; CASPR=contactin-associated protein-2; CRMP5=collapsin response-mediator protein-5; IgG=immunoglobulin G; LGI1=leucine-rich glioma-inactivated-1; mRS=modified Rankin Scale; NIS=neuropathy impairment score; PCA=Purkinje-cell cytoplasmic; VGCC=voltage gated calcium channel; VGKC=voltage-gated potassium channel complex ER -