Fatal Aβ cerebral amyloid angiopathy 4 decades after a dural graft at the age of 2 years
Intracerebral injection of brain homogenates containing misfolded Aβ can seed Aβ deposition in APP transgenic mice . 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 . 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 . 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...
The authors are grateful to the family of the patient that permitted the publication and helped the documentation of the clinical history. The role of the Brain Bank NeuroCEB funded by the patients’ Associations Vaincre Alzheimer, France Parkinson, Fondation ARSEP, Comprendre les Syndromes Cérébelleux, IHU A-ICM, and Fondation Plan Alzheimer is greatly acknowledged.
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