Stroke Research Centre, Department of Brain Repair and Rehabilitation, University College London Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery, London.
Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London.
Ann Neurol. 2019 Feb;85(2):284-290. doi: 10.1002/ana.25407. Epub 2019 Jan 17.
Amyloid-β transmission has been described in patients both with and without iatrogenic Creutzfeldt-Jakob disease; however, there is little information regarding the clinical impact of this acquired amyloid-β pathology during life. Here, for the first time, we describe in detail the clinical and neuroimaging findings in 3 patients with early onset symptomatic amyloid-β cerebral amyloid angiopathy following childhood exposure to cadaveric dura (by neurosurgical grafting in 2 patients and tumor embolization in a third). Our observations provide further in vivo evidence that cerebral amyloid angiopathy might be caused by transmission of amyloid-β seeds (prions) present in cadaveric dura and have diagnostic relevance for younger patients presenting with suspected cerebral amyloid angiopathy. Ann Neurol 2019; 1-7 ANN NEUROL 2019;85:284-290.
淀粉样蛋白-β在有和没有医源性克雅氏病的患者中都有传播；然而，关于这种获得性淀粉样蛋白-β病理学在生命过程中的临床影响的信息很少。在这里，我们首次详细描述了 3 名儿童时期接触过尸体硬脑膜（2 名患者通过神经外科移植物，1 名患者通过肿瘤栓塞）后出现早发性症状性淀粉样蛋白-β脑淀粉样血管病的患者的临床和神经影像学发现。我们的观察结果提供了进一步的体内证据，表明脑淀粉样血管病可能是由尸体硬脑膜中存在的淀粉样蛋白-β种子（朊病毒）传播引起的，并且对疑似脑淀粉样血管病的年轻患者具有诊断意义。Ann Neurol 2019; 1-7 ANN NEUROL 2019;85:284-290.
