Abstract Details

Title A Challenging Case of Cerebral Amyloid Angiopathy-related Inflammation (CAAri): Implications for Secondary Stroke Prevention and Immunomodulation Therapies

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 13-002

Objective

To present a case of cerebral amyloid angiopathy-related inflammation (CAAri), a rare CAA variant with unique clinical, histopathological, and imaging findings, and discuss management implications in the setting of ischemic stroke and adverse effects of immunotherapy.

Background CAAri is characterized by altered mentation, headaches, and seizures, as well as imaging findings of edema, cerebral microbleeds (CMBs), and inflammation. Histopathology shows non-destructive perivascular inflammation and amyloid deposition in vessel walls. Patients often require immunotherapy with steroids and steroid-sparing agents to prevent disease progression and relapse. One of the major causes of mortality in patients with CAAri is the associated risk of lobar intracerebral hemorrhage (ICH). This presents a challenge when treating CAAri patients with concomitant ischemic stroke, with a modicum of current evidence-based recommendations to guide management.

Design/Methods We retrospectively reviewed the chart of a patient with clinical, radiographic, and histopathologic findings consistent with CAAri.

Results A 65 year old male with no known cerebrovascular risk factors presented with 3 weeks of persistent headache, gait unsteadiness, and facial droop. MRI and CSF findings were consistent with diagnosis of CAAri. Brain biopsy showed vascular and perivascular amyloid deposition. He was started on steroids with prolonged taper given multiple relapses, and developed steroid-associated myopathy. He was started on cyclophosphamide for a 6 month treatment course. He remained clinically and radiographically stable until developing a left cerebellar ischemic stroke, etiology embolic stroke of undetermined source (ESUS). Clopidogrel was started for secondary stroke prevention.

Conclusions This case highlights the potential challenges associated with treating CAAri. In the setting of relapses and adverse side effects with steroids, steroid-sparing agents such as cyclophosphamide may be necessary for optimal outcomes. Additionally, secondary ischemic stroke prevention with agents such as clopidogrel or cilostazol may be better treatment options than aspirin given the increased risk of ICH in this patient population.

Authors/Disclosures
Joseph Nguyen, DO (Penn State Health Hershey Medical Center) PRESENTER	Dr. Nguyen has nothing to disclose.
Grant W. Zeigler, DO (Penn State Hershey Medical Center)	Dr. Zeigler has nothing to disclose.
Kerstin Bettermann, MD, PhD, FANA, FAAN (University of Pittsburgh Medical Center)	Dr. Bettermann has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bayer.
	No disclosure on file

��ɫ��

��ɫ��