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Abstract Details

Unilateral Cerebral Amyloid Angiopathy Presenting as Recurrent Right Hemispheric Hemorrhages
Cerebrovascular Disease and Interventional Neurology
P8 - Poster Session 8 (5:30 PM-6:30 PM)
Sporadic CAA is a common cause of intracerebral hemorrhage in older age groups that occurs due to β-amyloid deposition in cortical and subcortical blood vessel walls. There are scarce reports of CAA affecting a unilateral hemisphere.
To illustrate a rare presentation of cerebral amyloid angiopathy (CAA) with recurrent lateralized hemorrhages.
Case report.

A 60-year-old African American woman with a history of hypertension, chronic kidney disease, sleep apnea, and thrombocytopenia presented with recurrent right intraparenchymal hemorrhages since 2019. She initially presented with sudden onset headache and hypertensive urgency, neurologic examination was normal. Urgent neuroimaging showed an acute right temporo-parietal ICH with vasogenic edema and multiple right hemispheric microhemorrhages on SWI. Diagnostic angiogram did not show evidence of vascular malformation or vasculitis and malignancy screening was unrevealing. A surveillance MRI brain in 2020 showed two additional remote small hemorrhages in the right frontal and temporal lobes. 

She was admitted four times in 2022-2023 with recurrent headaches and seizures, with imaging showing new unilateral multifocal right hemispheric hemorrhages in the frontal, parietal, and temporal lobes with vasogenic edema, some in areas of known SWI change on prior MRI. She was treated with high dose steroids during two admissions without clear improvement. Two lumbar punctures revealed a bland CSF profile with normal infectious and autoimmune/inflammatory workup, cytology, cytometry, and normal CSF tau and Aβ42 levels. Due to diagnostic uncertainty with unilateral involvement, a right frontal lobe biopsy showed benign tissue that was negative for β-amyloid staining. Repeat right frontal lobe biopsy 6 months later showed occasional thickened vessel walls and focal intimal fibroplasia, and positive β-amyloid staining in multiple vessels confirming the diagnosis of definite CAA. 

Recurrent unilateral hemorrhages are a rare manifestation of CAA and should be considered in patients with lateralized cortical microhemorrhages in the absence of other clinical or diagnostic findings.
Maria Sokola, MD (Cleveland Clinic Main Campus)
Dr. Sokola has nothing to disclose.
Shervin Badihian, MD (Cleveland Clinic) Dr. Badihian has nothing to disclose.
Dolora R. Wisco, MD (Cleveland Clinic) Dr. Wisco has nothing to disclose.