Cerebral amyloid angiopathy related inflammation: three case reports and a review

Abstract

Cerebral amyloid angiopathy related inflammation (CAA-I), previously described under various names, is a treatable encephalopathy usually occurring in older adults. Here, three patients are described with histopathologically confirmed CAA-I, and summarised data from the published literature are presented. CAA-I has a characteristic combination of clinical and radiological features. Definite diagnosis requires brain and leptomeningeal biopsy. A favourable response to immunosuppressive therapy is common and treatment without brain biopsy may be considered in selected patients. Diagnostic criteria for CAA-I are proposed.

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…In cases in which genotyping has been performed, most patients have the apoe ε4/ε4 genotype [129]. CSF analysis may show elevated protein and/or pleocytosis [129].…”

“…In cases in which genotyping has been performed, most patients have the apoe ε4/ε4 genotype [129]. CSF analysis may show elevated protein and/or pleocytosis [129]. An MRI typically shows asymmetric white matter hyperintensities on T2 or FLAIR-weighted sequences, and in some cases, resembles a tumor [128].…”

“…Susceptibility weighted imaging sequences show evidence of prior hemorrhage (usually microhemorrhages) [129]. Cerebral angiography is generally normal, despite the fact that the pathology is due to vascular inflammation [129].…”

“…First line therapy is usually parenteral corticosteroids, and the response to corticosteroids is generally good [128,129,131,132]. In some cases, additional immunosuppressive agents, primarily cyclophosphamide, have been used [128].…”

Cerebral amyloid angiopathy: a transient ischaemic attack mimic

“…12 Another rare but important manifestation of CAA is CAA-related infl ammation (also known as cerebral amyloid angiitis, amyloid-β-related angiitis and cerebral amyloid infl ammatory vasculopathy). 13 CA A-related infl ammation generally affects older adults with acute/subacute cognitive decline, headache, behavioural change, seizures and focal neurological defi cits. 13 Magnetic resonance imaging (MRI) shows patchy or confl uent T2-weighted or fl uid-attenuated inversion recovery (FLAIR) white matter hyperintensities.…”