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

Autor: Ivo Bozovic, Marta Jeremic, Aleksandra Pavlovic, Carna Jovanovic, Nikola Kresojevic, Nikola Vojvodic, Dejana Jovanovic, Dragoslav Sokic, Milija Mijajlovic
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Brain Sciences, Vol 13, Iss 5, p 747 (2023)
Druh dokumentu: article
ISSN: 2076-3425
DOI: 10.3390/brainsci13050747
Popis: 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.
Databáze: Directory of Open Access Journals
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