RT期刊文章SR电子T1渐进多焦点的脑白质病与结节病:多中心病例系列摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP S69 OP S70 10.1212/01. wnl.0000903560.32562.13 VO 99 23补充2 A首页1迦勒R.S. McEntire A1安妮塔弗莱彻A1米歇尔•托莱达诺A1萨曼莎爱泼斯坦A1塞布丽娜Tan A1杨Mao-Draayer A1萨曼莎银行A1艾伦Aksamit A1杰弗里·m·盖尔芬德A1 Kiran Thakur A1艾琳Cortese A1经济部Bhattacharyya年2022 UL //www.ez-admanager.com/content/99/23_Supplement_2/S69.3.abstract AB目的我们的目标是描述的临床、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,旧金山加州大学,密歇根大学和贝斯以色列女执事医疗中心。结果25例患者与定S-PML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。 Of all patients, 16/25 patients had never received immunosuppressive medications prior to neurological symptom onset. Median serum lymphocyte count at time of PML diagnosis was 430 cells/uL (range: 50-1490). On MRI, 8/25 patients had infratentorial lesions only, while 8/25 had both infratentorial and supratentorial lesions, and 6/25 showed contrast enhancement. Seven/twenty-five patients progressed to death.Conclusions This study characterizes the clinical, laboratory, and imaging features of S-PML patients from seven major US medical centers. These data will be used to identify risk factors for development of PML in the context of sarcoidosis and to investigate any biomarkers that might aid in accurate and timely diagnosis.

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