Cerebral amyloid angiopathy related inflammation: A little known but not to be underestimated disease

Cerebral amyloid angiopathy related inflammation (CAA-ri) is a rare encephalopathy resulting from perivascular inflammation after β-βamyloid (A) deposition in cerebral vessels leading to progressive dementia, focal neurological signs, seizures and intracerebral hemorrhages. This condition is characterized on magnetic resonance imaging (MRI) by patchy or confluent T2/fluid attenuation inversion recovery (FLAIR) hyperintensities in the cortex and subcortical white matter located mainly in the same areas of pre-existing multiple microhemorrhages. In this report of 2 cases of “probable” CAA-ri women aged 71 and 68, we propose a review on the pathophysiological, clinical, radiological, therapeutic and prognostic aspects of this little-known and poor outcome condition. Even though an apparently favorable initial evolution after steroid and/or immunosuppressive treatment, CAA-ri course is unpredictable and often associated with low survival rates. We suggest the importance of timely and proper clinico-radiological evaluation in suspected CAA-ri cases, in order to start an appropriate treatment even without the brain biopsy.