@article {Dubeye1873作者= {Dubey Divyanshu和Jiraporn Jitprapaikulsan支Bi和Rocio巴斯克斯做南美草原和安德鲁·麦肯和肖恩·j·Pittock Janean k . Engelstad和约翰·r·米尔斯和克里斯托弗·j·克莱因},标题= {Amphiphysin-IgG自身免疫性神经病变},体积={93}={20},页面= {e1873——e1880} = {2019}, doi = {10.1212 / WNL。出版商0000000000008472}= {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={目的定义amphiphysin-immunoglobulin G的临床病理的特首页点(免疫球蛋白){\ textendash}介导神经病变。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) {\ textendash}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量表(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 {\ textendash}阳性病例。33(60 \ %)患者神经病变,21 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 \ %),扩散感官neuronopathy(35 \ %),与胃轻瘫和面部神经病变(3 \ %)。其中,疼痛(80 \ %),乳腺癌(63 \ %),对中枢神经系统(57 \ %)责任人通常共存,和神经病变常常引起乳腺癌的诊断(76 \ %)。Stiff-person谱系障碍是最常见的中枢神经系统伴奏(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}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/93/20/e1873}, eprint = {//www.ez-admanager.com/content/93/20/e1873.full.pdf}, journal = {Neurology} }