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Abstract Details

Cerebral Amyloid Angiopathy-Related Inflammation (CAA-ri) Masquerading as PRES
Cerebrovascular Disease and Interventional Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
5-004
To present a case of cerebral amyloid angiopathy-related inflammation mimicking posterior reversible encephalopathy syndrome, highlighting the diagnostic challenge and importance of early recognition.
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a distinct inflammatory subtype of cerebral amyloid angiopathy (CAA), characterized by an immune response targeting vascular beta-amyloid deposits. Clinically, CAA-ri may present with encephalopathy, headache, or seizures, and radiologically with asymmetric white matter changes and microhemorrhages. These features often overlap with Posterior Reversible Encephalopathy Syndrome (PRES), a condition marked by acute neurological symptoms and vasogenic edema, typically in the posterior brain regions and commonly associated with hypertension. This overlap poses a diagnostic challenge, as the management of these conditions differs significantly. PRES generally improves with correction of underlying triggers, while CAA-ri requires prompt immunosuppressive therapy. Delayed recognition of CAA-ri can lead to poor outcomes, underscoring the importance of early and accurate diagnosis.
N/A

A 75-year-old woman with hypertension, hyperlipidemia, and biopsy-proven CAA presented with acute encephalopathy. Head CT revealed bilateral parieto-occipital vasogenic edema, suggestive of PRES. Despite antihypertensive management, her mental status failed to improve.

Brain MRI showed diffuse, non-enhancing white matter hyperintensities, without diffusion restriction, and hemosiderin deposition – raising concern for CAA-ri. High-dose intravenous methylprednisolone and plasma exchange were initiated, but she showed no clinical improvement.

Due to refractory encephalopathy and progressive neurotoxicity, her family transitioned her to hospice care.
This case highlights the diagnostic overlap between PRES and CAA-ri and the importance of distinguishing between them. CAA-ri should be considered in PRES-like presentations, especially in normotensive patients or those with posterior-predominant edema, cognitive decline, or a history of CAA. A non-responsive or atypical course following PRES management should prompt further evaluation. Early immunosuppressive therapy is essential, though outcomes may be poor in advanced disease.
Authors/Disclosures
Christopher Gilbert, MD (Trinity Health Grand Rapids)
PRESENTER 		Dr. Gilbert has nothing to disclose.
Taleen Shahrigian, DO (Trinity Health Grand Rapids) Dr. Shahrigian has nothing to disclose.
Jason T. Schick, DO (Trinity Health Grand Rapids) Dr. Schick has nothing to disclose.
Lauren Turner, DO (Trinity Health Grand Rapids) Dr. Turner has nothing to disclose.
Muhammad Farooq, MD (Neuroscience Program,Saint Mary'S Health) Dr. Farooq has nothing to disclose.
Aileen Antonio, MD, FAAN (Trinity Health Saint Mary's Hauenstein Neurosciences) Dr. Antonio has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. An immediate family member of Dr. Antonio has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Giamarco, Mullins & Horton PC. The institution of Dr. Antonio has received research support from Saint Mary's Foundation. Dr. Antonio has received research support from 好色先生.