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

• Published in: Journal of clinical medicine Vol. 11; no. 22; p. 6731
• Main Authors: Theodorou, Aikaterini, Palaiodimou, Lina, Safouris, Apostolos, Kargiotis, Odysseas, Psychogios, Klearchos, Kotsali-Peteinelli, Vasiliki, Foska, Aikaterini, Zouvelou, Vasiliki, Tzavellas, Elias, Tzanetakos, Dimitrios, Zompola, Christina, Tzartos, John S, Voumvourakis, Konstantinos, Paraskevas, Georgios P, Tsivgoulis, Georgios
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 14-11-2022; MDPI
• Subjects: Biopsy; Case reports; Clinical medicine; Cohort analysis; Consent; Content analysis; Convulsions & seizures; Inflammation; Literature reviews; Magnetic resonance imaging; Medical imaging; Nervous system; Neuroimaging; Patients; Steroids; Stroke; Systematic review; microbleeds; Apolipoprotein E; MRI; focal deficits; inflammation; Cerebral Amyloid Angiopathy; FLAIR; steroids
• DOI: 10.3390/jcm11226731
• EISSN: 2077-0383

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.