Background and objectives: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a subtype of CAA with distinct clinical and radiologic features. Existing diagnostic criteria require the presence of characteristic asymmetrical white matter hyperintensity (WMH), together with classical hemorrhagic neuroimaging markers of CAA. There are limited data for other diagnostic neuroimaging markers of CAA-ri.
Methods: This is a case series from a specialist hospital intracerebral hemorrhage service.
Results: We describe 4 patients with CAA-ri who had regions of sulcal hyperintensity, with or without gyral swelling at clinical presentation, but did not fulfill current diagnostic criteria because of the absence of typical asymmetric WMH on brain MRI. All 4 patients were subsequently diagnosed with CAA-ri; three later developed asymmetric WMHs with disease relapse, and 2 had pathologically proven CAA-ri; 1 patient had both.
Discussion: Regions of sulcal hyperintensity, sometimes with associated gyral swelling, can be an early imaging finding in CAA-ri. These neuroimaging markers could potentially improve the accuracy of existing diagnostic criteria for CAA-ri to allow earlier diagnosis and treatment without biopsy in patients with atypical presentations.