文摘
客观的报告2例胶质原纤维酸性蛋白(GFAP) antibody-associated脑膜脑炎患者biopsy-proven系统性结节病。
背景GFAP astrocytopathy是一种自身免疫性神经疾病在2016年首次定义。据我们所知,没有与系统性结节病之前已经报道过了。
设计/方法案例系列
结果患者1是一个47岁的妇女与预先存在的肺结节病与类固醇和甲氨蝶呤治疗缓解6年前。她随后开发最近诊断为癫痫,进步运动失调和垂直复视。GFAP阳性抗体脑脊液(CSF)中细胞试验(CBA)。身体PET扫描显示弥漫性肺部FDG贪欲,脾脏、淋巴结,建议同时激活她的系统性结节病。她用类固醇治疗之后,英夫利昔单抗与解决她的症状。与已知的肺结节病病人2是一个58岁的男人,他是免疫抑制在演讲的时候但收到类固醇17年前。他提出了进步的冷漠,记忆障碍,构音障碍,步态不稳。MRI显示广泛的T2 hyperintensities。GFAP阳性抗体在CBA CSF和组织的免疫荧光试验确认。他收到了类固醇最初反应但类固醇停药后复发。 He improved after restarting steroids and was subsequently transitioned to infliximab with sustained neurologic recovery.
结论结节病是一种鲜为人知多系统紊乱,可能是一种免疫介导性应对未知的抗原(s)。众所周知共存与其他自身免疫性疾病、自身免疫性甲状腺炎是最常见的。GFAP astrocytopathy也知之甚少。GFAP发现细胞与细胞内抗原表位类似于其他抗体介入疾病,抗体被认为是潜在的自身免疫的生物标志物但不是直接致病。我们报告这些情况下强调鞘内GFAP的潜在关联生产抗体和系统性结节病,它可以提供洞察这两种疾病的发病机理。
脚注
披露:马修斯博士没有披露。博士手中获得了个人补偿的范围0 - 499美元的服务在演讲人生原体Idec。博士手中获得了个人的薪酬在500 - 4999美元的范围在默克的演讲人。博士手中获得了个人的薪酬在500 - 4999美元的范围扬声器Neurodiem管理局服务。Kammeyer博士的机构已收到个人薪酬在500 - 4999美元的范围为基因泰克公司担任顾问。Titulaer博士的机构已经收到了来自荷兰癫痫基础研究支持(NEF 14 - 18和19-08)。Titulaer博士的机构已经收到了来自中超贝林研究支持。Titulaer博士的机构已收到支持的联合研究。Titulaer博士的机构已收到来自荷兰的研究支持科学研究组织(ZonMW, Memorabel倡议和E-RARE UltraAIE)。Titulaer博士已经收到了研究机构的支持疗法。 The institution of Dr. Titulaer has received research support from Dioraphte (charity). The institution of Dr. Titulaer has received research support from Guidepoint Global LLC. The institution of Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Novartis. The institution of Dr. Piquet has received research support from Abbvie. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Litigative Consultant with US-Dept HHS/DICP.
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