Early-Onset Cerebral Amyloid Angiopathy, A Prion-Like Disease: Case Report and Literature Review

Abstract

Background: Cerebral amyloid angiopathy is an extremely rare pathology in young patients. In this group of patients, humanto-human amyloid-β transmission has been hypothesized, following neurosurgical procedures carried out years before. Case Description: We report the case of a 38-year-old woman who suffered repeated intracerebral hemorrhages caused by cerebral amyloid angiopathy three decades after an astrocytoma resection with cadaveric dural graft used for closing. Neuroimaging showed extensive left parenchymal hematoma, several microbleeds and frontal and occipital superficial siderosis. Pathology showed blood vessels with deposits stained with Congo Red, suggestive of cerebral amyloid angiopathy. Genetic tests did not reveal any mutation related to hereditary forms of β -amyloid pathology. Conclusion: This case report highlights the importance of considering cerebral amyloid angiopathy as part of differential diagnosis of lobar intracerebral hemorrhage in young adults, especially in those with a past medical history of neurosurgery and provide new evidence to support that amyloid-β might be transmitted as a prion disease.