Focal Cerebral Arteriopathy of the Young: Clinical Profile, Outcomes, and Utility of High-Resolution Vessel Wall Imaging.

INTRODUCTION: The data on focal cerebral arteriopathy (FCA) in young adults are limited to very few series, and the role of high-resolution magnetic resonance vessel wall imaging (MRVWI) has not been adequately explored.

METHODS: FCA was defined as unilateral, unifocal nonprogressive stenosis/irregularity of the distal internal carotid artery (ICA) or its proximal branches. All young patients aged 18-55 years admitted to our institute between 2010 and 2022 were retrospectively reviewed, and functional outcomes were quantified using the modified Rankin scale.

RESULTS: We identified 10 patients (9 males) with FCA giving an incidence rate of 2.8 per 1,000 person-years. Nine patients had an ischemic presentation (transient ischemic attacks = 5, minor strokes = 3, and major stroke = 1), while 1 patient had a cortical subarachnoid hemorrhage. The supraclinoid ICA and proximal middle cerebral artery (MCA) were the most common segments involved. Nine patients were treated with antiplatelets, and 3 received additional steroids. All cases underwent MRVWI, with concentric wall enhancement noted in most (80%). Follow-up imaging showed improvement in stenosis in 2 patients and a reduction in vessel wall enhancement in 3 patients. Except for the patient with a major stroke, all others had excellent short- and long-term outcomes with no recurrence of ischemic events. The presence of vessel wall enhancement did not predict short- or long-term outcomes.

CONCLUSION: FCA is a rare cause of young stroke that preferentially involves the supraclinoid ICA and proximal MCA with excellent outcomes. MRVWI can be useful in ruling out mimics, but the presence of contrast enhancement did not predict outcome.