Cerebral Amyloid Angiopathy -Related Inflammation (CAA-ri): Is Steroid/Imunosuppressive Therapy Beneficial?

Background: Cerebral amyloid angiopathy (CAA) is an important cause of intracerebral hemorrhage. Brain amyloid-related imaging abnormalities (ARIA) can be classified into two groups; those with edema or sulcal effacement (ARIA-E) and those with cerebral microbleeds or small hemorrhages (ARIA-H). There is an aggressive form of CAA, called CAA-related inflammation (CAA-ri) which is characterized by vascular or perivascular inflammation. Current treatment guidelines do not recommend routine use of steroids for intraparenchymal hemorrhage. Methods: We describe the clinical course, radiologic and pathologic findings of a 78-year-old man with recurrent intracerebral hemorrhage. Diagnostic studies included CT and MRI-scans of the brain, histopathology studies and autopsy. Results: The patient was diagnosed with a vasculitic form of amyloid angiopathy. Treatment with steroids resulted in clinical and radiological improvement. Conclusions: There might be a benefit of steroid or other immunosuppressive therapy in some patients with recurrent lobar hemorrhage related to CAA. Patients demonstrating edema out of proportion to the size of hematoma and extension of edema to the sub-cortical U fibers on imaging studies due to underlying vasculitis or CAA-ri may be candidates for such therapeutic interventions.