Teaching Neuroimages: Inflammatory CAA
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Inflammatory cerebral amyloid angiopathy (CAA) includes a spectrum of inflammatory diseases with similar clinical and imaging features, e.g. CAA-related inflammation (CAA-ri) and Aβ-related angiitis (ABRA). The CAA-ri is histologically characterized by focal accumulation of amyloid-β (Aβ) in leptomeningeal and parenchymal blood vessels associated with segmental perivascular inflammatory infiltrates and ABRA by the presence of intramural granulomas and regions of vessel wall destruction [ 1, 2]. Patients with inflammatory CAA typically present with rapid cognitive decline, focal neurologic deficits, headache, and/or seizures. Diagnostic criteria have recently been proposed for CAA-ri (Table 1; [ 5, 6]). The differential diagnosis of inflammatory CAA includes posterior reversible encephalopathy syndrome (PRES), acute disseminated encephalomyelitis (ADEM), reversible cerebral vasoconstriction syndrome (RCVS), chronic hypertensive encephalopathy, primary angiitis of the central nervous...
Conflict of interest
I.E. Duman, V.A. Coenen, S. Doostkam and H. Urbach declare that they have no competing interests.
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