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

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 dia...

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Autores principales: Bozovic, Ivo, Jeremic, Marta, Pavlovic, Aleksandra, Jovanovic, Carna, Kresojevic, Nikola, Vojvodic, Nikola, Jovanovic, Dejana, Sokic, Dragoslav, Mijajlovic, Milija
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10216068/
https://www.ncbi.nlm.nih.gov/pubmed/37239219
http://dx.doi.org/10.3390/brainsci13050747
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author Bozovic, Ivo
Jeremic, Marta
Pavlovic, Aleksandra
Jovanovic, Carna
Kresojevic, Nikola
Vojvodic, Nikola
Jovanovic, Dejana
Sokic, Dragoslav
Mijajlovic, Milija
author_facet Bozovic, Ivo
Jeremic, Marta
Pavlovic, Aleksandra
Jovanovic, Carna
Kresojevic, Nikola
Vojvodic, Nikola
Jovanovic, Dejana
Sokic, Dragoslav
Mijajlovic, Milija
author_sort Bozovic, Ivo
collection PubMed
description 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.
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spelling pubmed-102160682023-05-27 Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review Bozovic, Ivo Jeremic, Marta Pavlovic, Aleksandra Jovanovic, Carna Kresojevic, Nikola Vojvodic, Nikola Jovanovic, Dejana Sokic, Dragoslav Mijajlovic, Milija Brain Sci Case Report 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. MDPI 2023-04-29 /pmc/articles/PMC10216068/ /pubmed/37239219 http://dx.doi.org/10.3390/brainsci13050747 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Bozovic, Ivo
Jeremic, Marta
Pavlovic, Aleksandra
Jovanovic, Carna
Kresojevic, Nikola
Vojvodic, Nikola
Jovanovic, Dejana
Sokic, Dragoslav
Mijajlovic, Milija
Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title_full Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title_fullStr Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title_full_unstemmed Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title_short Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review
title_sort cerebral amyloid angiopathy-related inflammation (caa-ri): three heterogeneous case reports and a focused literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10216068/
https://www.ncbi.nlm.nih.gov/pubmed/37239219
http://dx.doi.org/10.3390/brainsci13050747
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