Cerebral Amyloid Angiopathy Associated with Inflammation: Report of 3 Cases and Systematic Review

Abstract

Introduction: Cerebral amyloid angiopathy associated with inflammatory process (CAA-I) is a rare potentially treatable encephalopathy, characterized by an inflammatory response to vascular deposits of β-amyloid. We aimed to describe 3 clinical cases and perform a systematic review of all neuropathologically proved CAA-I case reports to describe its clinical and pathologic features and outcome under different treatments.

Methods: We searched PubMed and Cochrane Library and screened references of included studies and review articles for additional citations. Outcome was classified at the last available follow-up by the modified Rankin Scale (mRS).

Results: A total of 67 publications, reporting on 155 patients, were included. Mean age was 66.9 years, and 53.5% were men. The most common clinical presentation was cognitive dysfunction (48.0%) followed by headaches (38.7%), seizures (36.7%), and pyramidal signs (20.0%). Perivascular and vasculitic inflammation with granuloma was the most common pathologic pattern (27.5%). Eighty-six percent were treated with corticosteroids and 33.9% with cyclophosphamide. Forty-two percent regained independence (mRS score 0-2), whereas 20.5% were left with a severe handicap (mRS score 3-5) and 37.5% died. There were no statistically significant differences in outcome between patients treated with therapy with corticosteroids alone comparing with those treated with combination corticosteroids with cytostatic agents.

Conclusions: The most common clinical manifestation of CAA-I was cognitive dysfunction. The functional outcome was unfavorable in the majority of the patients, with death or severe disability in almost two third of the cases, despite treatment. No differences in outcome could be detected between patients treated with corticosteroids versus patients treated with cytostatics, combined with corticosteroids.

Keywords: Cerebral amyloid angiopathy–related inflammation; amyloid angiopathy; brain biopsy; cerebral vasculitis; cytostatics; steroids.