A Night at the Met - Presentation PPT

Brain biopsy revealed gliosis, microglial activation, and histiocytic infiltration in addition to hemosiderin pigment and granulation tissue. Thickened vascular channels, many displaying a variably abundant mixed inflammatory infiltrate, were consistent with vasculitis. Amyloid-beta stains demonstrated widespread immunoreactivity within intraparenchymal and leptomeningeal blood vessels, with focal transmural involvement. Immunostains for amyloid-beta were positive. PCR tissue analysis, sent to the CDC, disclosed no infectious agents. A diagnosis of amyloid-beta-related angiitis (ABRA) was made. While cerebral amyloid angiopathy is common, rarely it can elicit an inflammatory response. There are two forms: inflammatory cerebral amyloid angiopathy, which is primarily perivascular, and the exceedingly rare amyloid-beta related angiitis (ABRA), which features frank vessel wall inflammation and granulomatous destruction. The amyloid deposits are thought to incite the vasculitic reaction. Regarded as a close variant or subtype of primary CNS angiitis, ABRA is histopathologically distinct in that amyloid co-localizes with inflammatory changes, a feature not seen in PACNS. Both conditions respond variably to immunotherapy and there is some suggestion of a better response to immunotherapy in ABRA. Our patient was treated with prednisone 60 mg/day for several weeks and transitioned to azathioprine. She has clinically improved with resolution of enhancement on MRI.