Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Center Experience and a Literature Review

Aikaterini Theodorou; Lina Palaiodimou; Apostolos Safouris; Odysseas Kargiotis; Klearchos Psychogios; Vasiliki Kotsali-Peteinelli; Aikaterini Foska; Vasiliki Zouvelou; Elias Tzavellas; Dimitrios Tzanetakos; Christina Zompola; John S Tzartos; Konstantinos Voumvourakis; Georgios P Paraskevas; Georgios Tsivgoulis

doi:10.3390/jcm11226731

Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Center Experience and a Literature Review

J Clin Med. 2022 Nov 14;11(22):6731. doi: 10.3390/jcm11226731.

Authors

Aikaterini Theodorou¹, Lina Palaiodimou¹, Apostolos Safouris², Odysseas Kargiotis², Klearchos Psychogios², Vasiliki Kotsali-Peteinelli¹, Aikaterini Foska¹, Vasiliki Zouvelou³, Elias Tzavellas⁴, Dimitrios Tzanetakos¹, Christina Zompola¹, John S Tzartos¹, Konstantinos Voumvourakis¹, Georgios P Paraskevas¹, Georgios Tsivgoulis^{1

5}

Affiliations

¹ Second Department of Neurology, "Attikon" University Hospital, School of Medicine, National and Kapodistrian University of Athens, 12462 Athens, Greece.
² Stroke Unit, Metropolitan Hospital, Ethnarhou Makariou 9, N. Faliro, 18547 Piraeus, Greece.
³ First Department of Neurology, "Aiginition" Hospital, School of Medicine, National and Kapodistrian University of Athens, 11528 Athens, Greece.
⁴ First Department of Psychiatry, "Aiginition" Hospital, School of Medicine, National and Kapodistrian University of Athens, 11528 Athens, Greece.
⁵ Department of Neurology, University of Tennessee Health Science Center, Memphis, TN 38163, USA.

Abstract

Background: Limited data exist regarding the prevalence of clinical, neuroimaging, and genetic markers among patients diagnosed with Cerebral Amyloid Angiopathy−related inflammation (CAA-ri). We sought to determine these characteristics in patients diagnosed in our center and to summarize available literature published either as single-case reports or small case series (<5 patients). Methods: We reported our single-center experience of patients diagnosed with CAA-ri according to international criteria during a seven-year period (2015−2022), and we abstracted data from 90 previously published cases. Results: Seven patients (43% women, mean age 70 ± 13 years) were diagnosed with CAA-ri in our center. The most common symptom at presentation was focal neurological dysfunction (71%), and the most prevalent radiological finding was the presence of T2/FLAIR white matter hyperintensities (100%). All patients were treated with corticosteroids and had a favorable functional outcome. Among 90 previously published CAA-ri cases (51% women, mean age 70 ± 9 years), focal neurological dysfunction was the most common symptom (76%), followed by a cognitive decline (46%) and headache (34%). The most prevalent neuroimaging findings were cerebral microbleeds (85%), asymmetric T2/FLAIR white matter hyperintensities (81%), and gadolinium-enhancing T1-lesions (37%). Genetic testing for the Apolipoprotein-E gene was available in 27 cases; 59% carried the APOE ε4/ε4 genotype. The majority of the published CAA-ri cases (78%) received corticosteroid monotherapy, while 17 patients (19%) were treated with additional immunosuppressive treatment. Favorable functional outcome following treatment was documented in 70% of patients. Conclusion: Improving the vigilance of clinicians regarding the early recognition and accurate diagnosis of CAA-ri is crucial for swift therapy initiation, which may result in improved functional outcomes.

Keywords: Apolipoprotein E; Cerebral Amyloid Angiopathy; FLAIR; MRI; focal deficits; inflammation; microbleeds; steroids.

Grants and funding

This research received no external funding.