文摘
客观的我们的目标是描述临床、实验室和影像学特征渐进多焦点的患者脑白质病(PML)在结节病(S-PML)。
背景结节病与CD4 +, CD8 +、CD19 +淋巴细胞减少,t细胞无力,增加感染的风险。S-PML已经报告了大约60例。PML经常被误认为是神经,导致有害的高剂量类固醇。初步证据显示,实验疗法如白细胞介素2和7、检查点抑制剂,polyomavirus-specific t细胞疗法,和英夫利昔单抗可能为治疗提供承诺。确保最优的结果,确定S-PML准确是至关重要的,以最小的延迟。
设计/方法回顾性搜集数据和成像对病人从电子病历质量General-Brigham网络医院、国立卫生研究院,梅奥诊所,欧文哥伦比亚大学医学中心,加州大学旧金山,密歇根大学和贝斯以色列女执事医疗中心。
结果25定S-PML患者确认。在结节病的诊断是54年,年龄中位数和结节病和PML诊断之间的平均时间为12个月。14/25的患者的肺结节病被隔离;10/25有多系统参与;一个病人有孤立的皮肤结节病。的病人,16/25的患者神经系统症状出现之前从未收到过免疫抑制药物。血清中淋巴细胞计数的PML诊断430细胞/ uL(范围:50 - 1490)。在MRI上,8/25患者infratentorial病变,而8/25都infratentorial和幕上的病变,和6/25显示对比度增强。7 / 25患者进展至死。
结论本研究描述的临床、实验室和成像特性S-PML病人从七个主要医疗中心。这些数据将被用来识别风险因素发展的PML的结节病和调查任何可能帮助的生物标记准确和及时的诊断。
脚注
披露:McEntire博士没有披露。弗莱彻博士没有披露。托莱达诺博士没有披露。爱泼斯坦博士没有披露。陈庆炎已收到个人薪酬在500 - 4999美元的范围为在科学咨询服务或数据安全监测委员会Cellevolve。陈庆炎的机构收到NIH的研究支持。Mao-Draayer博士已经收到个人薪酬在10000 - 49999美元的范围作为顾问生原体Idec。Mao-Draayer博士已经收到个人薪酬在500 - 4999美元的范围为EMD Serono担任顾问。Mao-Draayer博士已经收到个人薪酬在500 - 4999美元的范围为Genzyme-Sanofi担任顾问。Mao-Draayer博士已经收到个人薪酬在500 - 4999美元的范围为基因泰克公司担任顾问。 Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genzyme-Sanofi. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizone. Dr. Mao-Draayer has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen Idec. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Janssen. The institution of Dr. Mao-Draayer has received research support from NIH. The institution of Dr. Mao-Draayer has received research support from Genzyme-Sanofi. The institution of Dr. Mao-Draayer has received research support from Novartis. The institution of Dr. Mao-Draayer has received research support from Genentech. Dr. Banks has nothing to disclose. Dr. Aksamit has nothing to disclose. Dr. Gelfand has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. An immediate family member of Dr. Gelfand has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Headache: The Journal of Head and Face Pain. The institution of Dr. Gelfand has received research support from Genentech/Roche. The institution of an immediate family member of Dr. Gelfand has received research support from Amgen. The institution of Dr. Gelfand has received research support from Vigil Neurosciences. An immediate family member of Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant and Expert, Vaccine Injury Compensation Program with United States Health and Human Services and Department of Justice. Dr. Gelfand has a non-compensated relationship as a Trial Steering Committee with Roche / Genentech that is relevant to AAN interests or activities. Dr. Thakur has received personal compensation for serving as an employee of World Health Organization. The institution of Dr. Thakur has received research support from Center for Disease Control and Prevention. The institution of Dr. Thakur has received research support from National Institute of Health. The institution of Dr. Thakur has received research support from Biomerieux. Dr. Cortese has received stock or an ownership interest from Keires, AG. Dr. Cortese has received stock or an ownership interest from Nouscom, AG. Dr. Cortese has received stock or an ownership interest from PDC Pharma. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.
- ©2022美国神经病学学会的首页
