PC3079: Inflammatory Focal Cerebral Arteriopathy - An Important Cause of Childhood Stroke

Introduction: Although rare, affecting roughly 1-2 per 100,000 children, stroke remains among the top 10 causes of pediatric morbidity and mortality. Focal cerebral arteriopathy (FCA) is one of the most common causes of acute ischemic stroke in children who are previously healthy. A limited understanding of FCA and delays in its diagnosis result in inconsistent approaches to evaluation and treatment across centers.

Case Description: A 12 year old boy presented to the ED with sudden onset of dense left-sided weakness of the face, upper and lower extremities, right gaze deviation, confusion, and slurred speech. MRI Brain on admission revealed acute ischemic change in the right basal ganglia specifically involving the caudate and putamen. CTA Head and Neck showed filling defects in the distal right ICA and possible beading of the vessel. On day 3 of admission to the PICU and while on anticoagulation, he developed dense left hemiplegia, left lower facial weakness, and right gaze deviation. CTA demonstrated a second thrombus in the right M2 segment in addition to the thrombus in the distal right ICA, without signs of hemorrhage. He was administered tPA and taken for clot retrieval, with subsequent clinical improvement. About 24 hours post-procedure, he had acute worsening of his previous deficits, with repeat CTA re-demonstrating a filling defect in the right M1 segment. Laboratory investigations and prothrombotic labs were non-contributory. Due to worsening vascular disease restricted to the right-sided anterior circulation in the region of the distal ICA and MCA and irritability of the vessel during the cerebral angiogram, focal cerebral arteriopathy (FCA) was suspected and pulsed high-dose steroid therapy was started with subsequent clinical improvement.

Discussion: FCA accounts for approximately half of all cases of pediatric ischemic stroke and is a significant risk factor for both primary and recurrent stroke. It is a unifocal and unilateral stenosis/irregularity of the large intracranial arteries of the anterior circulation, and an important cause of all childhood arterial ischemic stroke particularly in children aged 8-15, who are previously healthy. There are two subtypes, namely inflammatory and dissection. i-FCA, the inflammatory subtype, may overlap with an infectious or post-infectious vasculopathy. VZV has been suggested to have a causal association with FCA. Early initiation of steroid therapy can help treat FCA and prevent stroke recurrence.

Conclusion: Among pediatric patients who present with symptoms of a stroke, FCA must be considered as a diagnosis and steroid therapy should be initiated promptly. Additional research is needed to determine the efficacy of adding pulsed dose steroids to pediatric acute stroke protocols.