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Amyloid-β (Aβ) is a peptide deposited in the brain parenchyma in Alzheimer’s disease and in the wall of small cortical and leptomeningeal vessels in cerebral amyloid angiopathy (CAA). Sporadic CAA is prevalent in an ageing population and is a major cause of intracerebral haemorrhage and dementia. There is evidence of Aβ transmission through medical procedures, such as injection of cadaver-derived growth hormone (GH) or cadaveric dura mater graft in humans, in the context of iatrogenic Creutzfeldt-Jakob disease (iCJD).1 Interestingly, there is increasing evidence to suggest that Aβ transmission may also occur in the absence of iCJD through neurosurgical procedures.2 3
This paper describes a case of early onset, pathologically proven CAA presenting with multiple symptomatic intracerebral haemorrhages occurring three decades after cardiac surgery involving a cadaveric dura mater patch.
A young male with multiple intracerebral haemorrhages underwent an extensive multimodal evaluation including clinical examination, cerebral MRI, amyloid positron emission tomography using 18F-florbetapir, apolipoprotein E (APOE) genotype, genetic testing for mutations or duplication causing hereditary CAA (amyloid precursor protein (APP), gelsolin, transthyretin and cystatin 3) and neuropathological examination of a brain biopsy. Sporadic CAA was confirmed pathologically. Childhood exposure to cadaveric dura mater was suspected based on the surgical history for congenital heart disease, then confirmed. The patient’s informed consent was obtained.
A man aged 34 years presented with multiple symptomatic lobar intracerebral haemorrhages. He had a medical history of transposition of the great arteries, requiring neonatal cardiac surgery with the Rashkind procedure. At 2 years of age, the patient underwent repeat surgery with a Senning …
Twitter @Mélanie Planton, @Francois Chollet
Contributors NR: design and conceptualised study; supervised the study; collected the data; analysed and interpreted the data; wrote the manuscript; revised the manuscript; prepared the figures. AS and EU-C: analysed and interpreted the data; revised the manuscript; prepared the figures. MP, LC, PP, J-FA, AV, M-BD, FC, FB and J-MO: analysed and interpreted the data; revised the manuscript. JP: design and conceptualised study; analysed and interpreted the data; revised the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests NR was supported by a Fulbright Scholarship, and received an Arthur Sachs Scholarship from the Harvard University Committee on General Scholarship, and a Philippe Foundation research grant. PP reports personal fees from Lilly/Avid and from GE Healthcare. JP has received speaker honoraria from Lilly, Roche and Novartis.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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