Objective:

To report a case of presumed ipsilateral hemispheric cerebral amyloid angiopathy (CAA) after prior cadaver dural graft.

Background:

CAA is characterized by deposition of amyloid beta in the walls of cortical and leptomeningeal blood vessels. This deposition leads to increased fragility of the blood vessels and in turn recurrent hemorrhage. Recent studies have hypothesized that misfolded amyloid beta protein, like perhaps Creutzfeldt Jakob prions, may propagate in a new host after implantation with affected tissue.

Design/Methods:

Patient interview and chart review.

Results:

A 63-year-old man presented with recurrent multifocal right hemispheric lobar hemorrhages. History was notable for severe headaches at 20 years of age, which led to identification of a right frontal cyst resected with a homologous dural graft implant. Pathology of the resected lesion was consistent with an old arteriovenous malformation. He was then asymptomatic for 40 years until he developed left hemiplegia in the setting of an acute right frontal intraparenchymal hemorrhage (remote from area of prior resection), then three years later developed severe headaches in the setting of an acute right parieto-occipital hemorrhage, and subsequently underwent surveillance MRI imaging including susceptibility weighted imaging three months later which demonstrated asymptomatic hemorrhages of the right occipital lobe and right temporoparietal junction. Imaging demonstrated extensive siderosis involving nearly the entirety of the right cerebral hemisphere with sparing of the left hemisphere most consistent with CAA. CSF studies were positive for Alzheimer’s biomarkers (elevated p-Tau/Abeta42 ratio) but was otherwise unremarkable. APOE testing was e3/e3 (which is not associated with vasculopathy). Conventional angiogram was unremarkable and demonstrated no underlying cerebrovascular abnormality.

Conclusions:

Prior surgery with cadaver grafts may be a rare risk factor for the development of CAA. This case suggests that there may be direct seeding of amyloid beta from donor tissue and thus may provide insight into the pathophysiology of CAA.