We herein report that the clinical, laboratory, and radiographic features and positron emission tomography (PET) imaging may provide valuable clues to the pathogenesis of cerebral amyloid angiopathy (CAA)-associated encephalopathy, which currently remains unclear. We herein describe two cases of encephalopathy with CAA, with an emphasis on PET imaging with (11)C-Pittsburgh compound B ((11)C-PiB) and (18)F-fluorodeoxyglucose ((18)F-FDG). One case of Alzheimer's disease for which a brain biopsy was performed showed CAA-related inflammation. Another case that had developed sudden sensory aphasia presented with posterior reversible encephalopathy syndrome-like vasogenic edema in the left temporal region with (11)C-PiB uptake and microhemorrhages. (11)C-PiB and (18)F-FDG PET are useful for detecting CAA-associated encephalopathy, including atypical CAA cases.
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