Focal Cerebral Arteriopathy: The Face With Many Names

• Published in: Pediatric neurology 2015-09, Vol.53 (3), p.247-252
• Main Authors: Tolani, Alisha T, Yeom, Kristen W., MD, Elbers, Jorina, MD, MS
• Format: Article
• Language: English
• Subjects: Adolescent; Anterior Cerebral Artery - pathology; Brain Ischemia - complications; Brain Ischemia - diagnosis; Brain Ischemia - pathology; Carotid Artery Diseases - complications; Carotid Artery Diseases - diagnosis; Carotid Artery Diseases - pathology; carotid artery dissection; Carotid Artery, Internal - pathology; Cerebral Angiography; Cerebral Arterial Diseases - complications; Cerebral Arterial Diseases - diagnosis; Cerebral Arterial Diseases - pathology; Child; Diagnosis, Differential; Female; focal cerebral arteriopathy; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Middle Cerebral Artery - pathology; moyamoya disease; Neurology; pediatric stroke; Pediatrics; Retrospective Studies; Stroke - complications; Stroke - diagnosis; Stroke - pathology
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2015.05.008

Abstract Objective Focal cerebral arteriopathy is a term used to describe unilateral intracranial arteriopathy involving the distal internal carotid artery and proximal segments of the middle and anterior cerebral artery. We describe the disease course of 10 pediatric arterial ischemic stroke patients with focal cerebral arteriopathy from a single quaternary-care center. Methods We retrospectively reviewed pediatric stroke patients with focal cerebral arteriopathy without lenticulostriate collaterals treated at our institution between 2005 and 2014. Angiography was reviewed by a child neurologist and a pediatric neuroradiologist, and chart reviews were performed. Results Ten individuals with focal cerebral arteriopathy were identified. At the time of stroke presentation, four patients were diagnosed with arterial dissection, two with moyamoya disease, one with embolic occlusion, one with hemorrhagic stroke, and two with arterial dissection or vasculitis. At last follow-up, six patients had a change in diagnosis: four were diagnosed with transient cerebral arteriopathy, two with arterial dissection, and four with moyamoya disease. Four children experienced stroke recurrence. All were administered aspirin, one was administered heparin, two were administered intravenous tissue plasminogen activator, and five underwent surgical revascularization. Conclusions Among pediatric stroke patients with a similar angiographic appearance, there is variable concordance between diagnosis, prognosis and treatment choice. Improved consensus-based diagnostic criteria and further research is needed to identify disease biomarkers and predictors of arterial progression.