Abstract
BACKGROUND: Cerebral amyloid angiopathy (CAA) is a neurological condition characterized by the deposition of amyloid beta particles within the cerebral vasculature over time. A rare complication of CAA is an autoimmune inflammatory syndrome to cerebrovascular amyloid deposits. In this report, we present a case of CAA-related inflammation (CAA-RI) and discuss the diagnostic and management considerations when encountering this pathology. CASE DESCRIPTION: A 69-year-old man with a history of hypertension, hyperlipidemia, obstructive sleep apnea, benign prostatic hyperplasia, and major depressive disorder presented to the clinic with rapidly progressive cognitive impairment over the preceding 2 months. Magnetic resonance imaging (MRI) of the brain demonstrated white matter hyperintense lesions associated with innumerable microbleeds asymmetrically concentrated in the right parietal lobe, with subtle hyperattenuation of the sulci. These findings suggested a diagnosis of probable CAA-RI. The patient was started on intravenous methylprednisolone, one gram daily for 5 days, followed by a prolonged prednisone taper over the next 6 weeks. CONCLUSION: Patients with CAA-RI typically present with cognitive decline, followed by focal neurologic deficits, seizures, and headaches. On MRI of the brain, T2/fluid-attenuated inversion recovery asymmetric hyperintense white matter lesions local to cerebral microbleeds are characteristic. Management of CAA-RI involves high-dose corticosteroids with emerging investigation of immunosuppressive therapies.