Abstract WP483: Grouped Cortical Micro Bleeds a Unique Radiological Marker in Inflammatory Cerebral Amyloid Angiopathy

Abstract

Background: Inflammatory Cerebral Amyloid Angiopathy (iCAA) is a rare cause of rapidly progressive cognitive decline. Prior studies have proposed radiological diagnostic criteria and demonstrated evidence of inflammation related to Aβ. Though lacking any therapeutic trials, various authors have suggested a single pulse IV Methyl Prednisolone (MPS) therapy as curative in most patients. Methods: We studied clinical, radiological and neuropathological features of 2 of biopsy proven iCAA cases over the past 5 years correlating their neuropathological and radiological features. We also reviewed published literature for radiological findings of proven cases of ICAA Clinical features Case 1: 75/female presented with visual hallucinations, her neurological examination was un-remarkable >Case 2: 84/female presented with language and memory difficulties her neurological examination was remarkable for word finding and object naming difficulties Radiological Features: Gadolinium enhanced brain MRI with fluid attenuated inversion recovery (FLAIR) and susceptibility weighted imaging (SWI) were obtained Patient 1: SWI artifact s in bilateral cerebral hemispheres basal ganglia and cerebellum suggestive of cortical micro-bleeds (CMBs ) there was a cluster of CMBs in the right parietal region associated with FLAIR changes and gadolinium enhancement Patient 2 numerous scattered cortical micro-bleeds (CMBs). There was a large cluster of CMBs in left temporal lobe along with FLAIR changes and gadolinium enhancement Biopsy: B oth patient underwent lesional biopsy corresponding to the area of clustered CMBs Neuropathology: demonstrated histopathological and electron microscopic evidence of perivascular inflammation and amyloid deposition consistent with iCAA Review of literature : In a total of 7 brain MRI s in 3 recently published case series 4 had reported CMBs of which 3 patients were identified representing MRI findings consistent with Grouped CMBs associated with FLAIR changes Conclusion: iCAA is a rare and treatable cause of subacute cognitive decline. We suggest grouped cortical micro bleeds (GCMBs) associated with FLAIR changes as a unique radiological feature of this disorder.