Meningovascular inflammation in cerebral amyloid angiopathy-related cortical superficial siderosis

The role of inflammation in cortical superficial siderosis (cSS), a marker of cerebral amyloid angiopathy (CAA) linked to high hemorrhage risk, is unclear. We examined 14 patients with cSS using 3T post contrast vessel wall MRI (VWI) and CSF analysis. Although only 31% met current CAAri criteria, 86% showed vessel wall enhancement or sulcal hyperintensities near cSS, frequently extending beyond. Four patients with follow up VWI demonstrated corticosteroid responsive regression of inflammation. CSF albumin quotients, indicating blood brain barrier dysfunction, correlated with MRI inflammation scores. These findings reveal subclinical meningovascular inflammation in cSS and support VWI for detecting a broader CAA related inflammation spectrum.