Inserm U1127, CNRS UMR 7225, UPMC Univ Paris VI UMR S 1127, Institut du Cerveau et de la Moelle épinière, Sorbonne Universités, 47 boulevard de l'Hôpital, 75013, Paris, France.
Laboratoire de Neuropathologie R. Escourolle, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France.
Acta Neuropathol. 2018 Feb;135(2):201-212. doi: 10.1007/s00401-017-1791-x. Epub 2017 Nov 22.
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.
对 24 名在 20 世纪 80 年代因接触尸体来源的人类生长激素(c-hGH)而死于医源性克雅氏病的法国患者进行了研究,以检测β淀粉样蛋白沉积和 tau 病理学变化。仅在一例潜伏期最长的病例中发现了β淀粉样蛋白沉积。有 3 例还存在细胞内 tau 积累。对 1974 年至 1988 年间生产的 24 批 c-hGH 进行的分析首次证明了 c-hGH 中存在 Abeta 和 tau 污染物(分别为 17 批和 6 批)。与之前发表的两个英国系列研究相比,法国患者的朊病毒病潜伏期较短。我们将法国系列中 Abeta 的低发生率解释为与英国相比,法国观察到的潜伏期较短所致。这一概念表明,在首次接触后 18 年以上,可检测到 Abeta 沉积的平均潜伏期会更长。此外,我们假设 tau 病理学也可能在人类中传播。