PT -期刊文章盟Dubey Divyanshu盟Jiraporn Jitprapaikulsan AU -支Bi盟Rocio巴斯克斯做Campo盟——安德鲁·麦肯AU -肖恩·j·Pittock盟Janean k Engelstad AU -约翰·r·米尔斯盟克里斯托弗·j·克莱因TI - - 10.1212 / WNL Amphiphysin-IgG自身免疫性神经病变援助。0000000000008472 DP - 2019年11月12日TA -神经首页病学PG - e1873 e1880 VI - 93 IP - 20 4099 - //www.ez-admanager.com/content/93/20/e1873.short 4100 - //www.ez-admanager.com/content/93/20/e1873.full所以Neurology2019 11月12;93 AB -目标定义的临床病理的特点amphiphysin-immunoglobulin G(免疫球蛋白)介导的神经病变。方法患者检查在我们的机构从1月1日,1995年9月30日,2018年,与amphiphysin-IgG通过间接免疫荧光和免疫印迹,综述。相比他们的表型的共存collapsin response-mediator protein-5 (CRMP5)免疫球蛋白或anti-neuronal核1型(ANNA1-IgG)和CRMP5-IgG自身免疫抗体。改善改良Rankin规模(夫人)(≥1)后续被认为是一个有利的结果。Amphiphysin RNA表达在健康评估神经。结果53 amphiphysin-IgG-positive病例。神经病变患者33例(60%),21个amphiphysin-IgG孤独,和12个共存自身抗体(ANNA1-IgG n = 8;CRMP5-IgG, n = 2;ANNA1-IgG CRMP5-IgG, n = 2)。在孤立amphiphysin-IgG自身免疫系统疾病包括多神经根神经病(62%)、弥漫性感官neuronopathy(35%),与胃轻瘫和面部神经病变(3%)。 Among these, pain (80%), breast cancer (63%), and CNS (57%) involvements commonly coexisted, and neuropathy frequently prompted breast cancer diagnosis (76%). Stiff-person spectrum disorder was the most common CNS accompaniment (45%). Nerve biopsies showed axonal loss (n = 6/6), subperineurial edema (n = 4/6), and CD4 predominant inflammation (n = 2/6). Median mRS score at last follow-up was 3.5; 58% of patients were immunotherapy-responsive. Patients with amphiphysin-IgG alone had more favorable immunotherapy response than patients with CRMP5-IgG polyneuropathy (n = 45) (44% vs 16%, p = 0.028, odds ratio 4.2, 95% confidence interval 1.1 to 15.5). Only 1/9 (11%) patients with amphiphysin-IgG with coexisting CRMP5-IgG or ANNA1-IgG had immunotherapy response. RNA amphiphysin expression occurred at low levels in nerve.Conclusion Amphiphysin-IgG autoimmune neuropathy has a recognizable phenotype, is frequently immune responsive, and can prompt early diagnosis of breast cancer.ANNA1=antineuronal nuclear antibody type 1; CRMP5=collapsin response-mediator protein-5; IgG=immunoglobulin G; IIF=indirect immunofluorescence; mRS=modified Rankin Scale; PBS=phosphate-buffered saline; RT=room temperature