SHM Converge Scientific Abstract ePoster Gallery-Not Your Typical Headache: Cerebral Amyloid Angiopathy-Related Inflammation

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This case describes a 57-year-old man with no prior headache history who developed 1 month of worsening bifrontal headaches, nausea, photophobia, word-finding difficulty, and progressive memory and cognitive decline. On exam, he was markedly hypertensive and had delayed speech initiation. Laboratory studies and CSF were largely unrevealing. Brain MRI showed asymmetric T2/FLAIR hyperintensity in the right temporal-parieto-occipital region, scattered microhemorrhages, and leptomeningeal/vascular enhancement, without evidence of vasculitis on vessel wall imaging.<br /><br />Based on the clinical picture and imaging, he was diagnosed with cerebral amyloid angiopathy-related inflammation (CAA-ri), a rare inflammatory small-vessel disorder caused by immune reaction to amyloid deposits in cortical or leptomeningeal vessels. He was treated with high-dose IV methylprednisone followed by a planned oral taper, but steroids were stopped early due to steroid-induced mania. His blood pressure was also controlled more aggressively. Follow-up MRI 2 months later showed marked improvement, and his symptoms improved clinically.<br /><br />Key teaching points: CAA-ri often presents subacutely with headaches, cognitive decline, behavioral change, or seizures. Brain biopsy is the diagnostic gold standard but is often avoided because it is invasive; MRI-based clinical criteria are commonly used and have high sensitivity and specificity. Typical MRI findings include asymmetric white matter hyperintensities, leptomeningeal enhancement, and microbleeds. First-line treatment is usually high-dose corticosteroids followed by a taper, with repeat imaging in 4–6 weeks to assess response. Relapses are common, and some patients require long-term immunosuppression. Recognition is increasingly important because amyloid-targeting therapies may trigger similar inflammatory reactions.

Asset Subtitle

Victoria Kalinoski-DuBose

Meta Tag

Author List Bibek Saha, Darin Carabenciov, Deborah Setter, Joelle N. Friesen, Victoria Kalinoski-DuBose

Category Clinical Vignettes

Concept Cerebral Amyloid Angiopathy-Related Inflammation

Concept Magnetic Resonance Imaging

Concept Brain biopsy

Concept Corticosteroid

Concept Headache

Distinguished Non-Finalist

Presenter Organization Mayo Clinic Rochester, MN

Presenting Author Victoria Kalinoski-DuBose

Track Adult

Keywords

cerebral amyloid angiopathy-related inflammation

CAA-ri

subacute headache

cognitive decline

MRI hyperintensity

microhemorrhages

leptomeningeal enhancement

corticosteroid treatment

brain biopsy

amyloid-related inflammation

Cerebral Amyloid Angiopathy-Related Inflammation

Magnetic Resonance Imaging

Brain biopsy

Corticosteroid

Headache