Clinical Presentation and Imaging Findings of Irreversible Cerebral Amyloid Angiopathy-Related Inflammation (CAA-ri): A Case Report and Literature Review

TL;DR

This case report describes a rare brain condition called CAA-ri, highlighting its symptoms, imaging features, and poor response to treatment.

Contribution

The paper presents a detailed case of irreversible CAA-ri and evaluates the timing of corticosteroid treatment.

Findings

The patient showed progressive neurological decline despite corticosteroid treatment.

Imaging revealed asymmetric cerebral white matter lesions linked to angioedema.

The case highlights the importance of early diagnosis for CAA-ri.

Abstract

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare condition primarily driven by an autoimmune reaction against cerebrovascular amyloid beta protein. Accurate diagnosis hinges on recognizing characteristic clinical symptoms and imaging features, such as asymmetric cerebral white matter lesions often linked to angioedema. We report the case of a woman in her 70s with progressive, irreversible CAA-ri who initially presented with left homonymous hemianopia and experienced significant psychiatric and neurological deterioration following an epileptic seizure. Despite initiating corticosteroid therapy seven months after onset, her condition continued to worsen, ultimately leading to her death in the 11th month due to general decline. This report reviews the clinical progression and imaging findings of the case, discusses the diagnostic process for CAA-ri, differentiates it from related conditions, and evaluates the timing of corticosteroid treatment.

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