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Neuropathology of iatrogenic Creutzfeldt–Jakob disease and immunoassay of French cadaver-sourced growth hormone batches suggest possible transmission of tauopathy and long incubation periods for the transmission of Abeta pathology
Acta Neuropathologica ( IF 12.7 ) Pub Date : 2017-11-22 , DOI: 10.1007/s00401-017-1791-x
Charles Duyckaerts , Véronique Sazdovitch , Kunie Ando , Danielle Seilhean , Nicolas Privat , Zehra Yilmaz , Laurène Peckeu , Elodie Amar , Emmanuel Comoy , Aleksandra Maceski , Sylvain Lehmann , Jean-Pierre Brion , Jean-Philippe Brandel , Stéphane Haïk

Abeta deposits and tau pathology were investigated in 24 French patients that died from iatrogenic Creutzfeldt–Jakob disease after exposure to cadaver-derived human growth hormone (c-hGH) in the 1980s. Abeta deposits were found only in one case that had experienced one of the longest incubation periods. Three cases had also intracellular tau accumulation. The analysis of 24 batches of c-hGH, produced between 1974 and 1988, demonstrated for the first time the presence of Abeta and tau contaminants in c-hGH (in 17 and 6 batches, respectively). The incubation of prion disease was shorter in the French patients than the incubation times reported in two previously published British series. We interpreted the low incidence of Abeta in this French series as a consequence of the shorter incubation period observed in France, as compared to that observed in the United Kingdom. This concept suggested that a mean incubation period for the development of detectable Abeta deposits would be longer than 18 years after the first exposure. Moreover, we hypothesized that tau pathology might also be transmissible in humans.



中文翻译:

医源性Creutzfeldt–Jakob病的神经病理学和法国尸体来源的生长激素批次的免疫测定表明,牛磺酸病可能会传播,而Abeta病理的潜伏期较长

在1980年代,对24名法国人死于医源性Creutzfeldt–Jakob病死于尸体来源的人类生长激素(c-hGH)后,对其Abeta沉积物和tau病理进行了调查。仅在经历了最长潜伏期之一的情况下才发现Abeta沉积物。三例也有细胞内tau积累。对1974年至1988年之间生产的24批次c-hGH的分析首次证明了c-hGH中分别存在Abeta和tau污染物(分别为17和6批次)。在法国患者中,ion病毒疾病的潜伏期比在先前发表的两个英国系列中报道的潜伏期短。我们认为,由于在法国观察到的潜伏期较短,因此该法国丛书中Abeta的发生率较低,与在英国观察到的相比。这个概念表明,第一次可暴露的Abeta沉积物发展的平均潜伏期将超过18年。此外,我们假设tau病理学也可能在人类中传播。

更新日期:2017-11-22
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