Kretzschmar Hans A, Sethi Shneh, Földvári Zsuzsanna, Windl Otto, Querner Veronika, Zerr Inga, Poser Sigrid
Institut für Neuropathologie, Ludwig-Maximilians-Universität München, Germany.
Brain Pathol. 2003 Jul;13(3):245-9. doi: 10.1111/j.1750-3639.2003.tb00025.x.
Florid plaques indistinguishable from those found in vCJD were identified at a postmortem examination in the brain of a 58-year-old clinical suspect case of Creutzfeldt-Jakob disease (CJD). Western blotting of brain tissue revealed an unusual prion protein type. Since the patient had received a dura mater graft 20 years prior to death and florid plaques are not only found in new variant CJD, the findings argue in favor of an iatrogenic origin of the disease with the longest incubation time following a dura mater graft reported to date even though he may have been exposed to BSE. The peculiar pathological, clinical and biochemical features may define a new type of human prion disease.
在一名58岁克雅氏病(CJD)临床疑似病例的尸检中,发现了与变异型克雅氏病(vCJD)中所见难以区分的显著斑块。脑组织的蛋白质印迹分析显示出一种不寻常的朊病毒蛋白类型。由于该患者在死亡前20年接受了硬脑膜移植,且显著斑块不仅见于新型变异型克雅氏病,这些发现支持了该病的医源性起源,这是迄今为止报道的硬脑膜移植后潜伏期最长的病例,尽管他可能接触过牛海绵状脑病。其独特的病理、临床和生化特征可能定义了一种新型人类朊病毒疾病。