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Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review

Authors :
Ivo Bozovic
Marta Jeremic
Aleksandra Pavlovic
Carna Jovanovic
Nikola Kresojevic
Nikola Vojvodic
Dejana Jovanovic
Dragoslav Sokic
Milija Mijajlovic
Source :
Brain Sciences, Vol 13, Iss 5, p 747 (2023)
Publication Year :
2023
Publisher :
MDPI AG, 2023.

Abstract

Cerebral amyloid angiopathy-related inflammation (CAA-rI) is a largely reversible, subacute encephalopathy, which is considered as a rare variant of cerebral amyloid angiopathy (CAA). Although the diagnosis of this inflammatory vasculopathy is generally clinico-pathologic, a probable or possible diagnosis can often be established based on current clinico-radiological diagnostic criteria. This is important since CAA-rI is considered as a treatable disorder, which most commonly occurs in the elderly population. Behavioral changes and cognitive deterioration are highlighted as the most common clinical signs of CAA-rI, followed by a heterogeneous spectrum of typical and atypical clinical presentations. However, despite the well-established clinical and radiological features incorporated in the current diagnostic criteria for this CAA variant, this rare disorder is still insufficiently recognized and treated. Here, we have shown three patients diagnosed with probable CAA-rI, with significant heterogeneity in the clinical and neuroradiological presentations, followed by different disease courses and outcomes after the introduction of immunosuppressive treatment. Moreover, we have also summarized up-to-date literature data about this rare, yet underdiagnosed, immune-mediated vasculopathy.

Details

Language :
English
ISSN :
20763425
Volume :
13
Issue :
5
Database :
Directory of Open Access Journals
Journal :
Brain Sciences
Publication Type :
Academic Journal
Accession number :
edsdoj.6d2c68c3816f4f86acdb2de981b6b4b3
Document Type :
article
Full Text :
https://doi.org/10.3390/brainsci13050747