Cerebral Amyloid Angiopathy: New Insights and Perspective

Sporadic cerebral amyloid angiopathy is a common neuropathological finding in the aging brain. It is caused by accumulation of amyloid-beta peptide in the leptomeningeal arteries, cortical arterioles, and capillaries, likely because of impaired cerebral clearance of amyloid-beta with increasing age. The study of rare hereditary forms of the disease suggests that a long pre symptomatic time is probable. The relation between CAA and high risk of recurrence of lobar intracerebral haemorrhage is widely recognized. Cognitive decline with an increased risk of dementia, and transient focal neurological episodes were described more recently. The existence of a rare and complex inflammatory form suggests the possibility of a specific autoimmune response. Biomarkers identification has improved our knowledge of natural history and recurrence risk of the disease: MRI (microbleeds, cortical micro-infarctions, cortical hemosiderosis, focal subarachnoid haemorrhage, cortical atrophy, white matter hyper signals, enlargement of perivascular spaces), Amyloid PET, CSF biomarkers contribute to characterize patients profile and risk. Despite the lack of neuropathology most of the time, MRI biomarkers provide a reliable probability for diagnosis (modified Boston Criteria). No specific therapy is currently available. However, many developments are in progress ongoing rapidly. Strict control of blood pressure, discussion of anticoagulant therapy in high embolic risk patients are key elements of the current care management of patients. (C) 2020 Published by Elsevier Masson SAS on behalf of l'Academie nationale de medecine.