Early onset cerebral amyloid angiopathy following childhood exposure to cadaveric dura
Early onset cerebral amyloid angiopathy following childhood exposure to cadaveric dura
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DOI：
10.1002/ana.25407
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发表时间：
2019-02-01
影响因子：
11.2
通讯作者：
Werring, David J.
中科院分区：
文献类型：
--
作者：
Banerjee, Gargi;Adams, Matthew E.;Werring, David J.
淀粉样蛋白β传播在有和没有医源性Creutzfeldt-Jakob病的患者中均有描述;然而，关于这种获得性淀粉样蛋白β病理学在生活中的临床影响的信息很少。在这里，我们第一次详细描述了3例儿童期暴露于尸体硬脑膜（2例神经外科移植，1例肿瘤栓塞）后早发症状性淀粉样β脑淀粉样血管病患者的临床和神经影像学表现。我们的观察提供了进一步的体内证据，脑淀粉样血管病可能是由尸体硬脑膜中存在的淀粉样β种子（朊病毒）的传播引起的，并对疑似脑淀粉样血管病的年轻患者具有诊断相关性。Ann Neurol 2019; 1-7 ANN Neurol 2019;85：284-290。
Amyloid-beta 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-beta 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-beta 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-beta 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.