Fatal Aβ cerebral amyloid angiopathy 4 decades after a dural graft at the age of 2 years

Excerpt

Intracerebral injection of brain homogenates containing misfolded Aβ can seed Aβ deposition in APP transgenic mice [7]. Parenchymal and vascular amyloid deposits have been observed in iatrogenic Creutzfeldt–Jakob disease (iCJD) caused by injection of cadaver-derived growth hormone (cGH) [1, 2, 5, 10]. This observation has raised the hypothesis that hormone preparations were not only contaminated with misfolded prion protein but also with Aβ aggregates able to seed amyloid formation; Aβ aggregates were, indeed, found in cGH batches produced at the time of the contamination [2]. Dural grafts, up to now associated with iCJD, have also been suspected to seed Aβ accumulation mainly in the vessel walls [3, 4, 6, 9]. Seeded Aβ aggregation, however, has not been previously associated with clinical manifestations [1]. We report here the case of a 46-year-old woman who died of repeated intracerebral hemorrhages caused by cerebral amyloid angiopathy (CAA), presumably related to contaminated cadaveric dura, grafted 44 years before death. …