title: Focal Cerebral Arteriopathy: The Face With Many Names.
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.
authors.affiliation: School of Medicine, Stanford University, Stanford, California.
authors.forename: Alisha T
authors.initials: AT
authors.lastname: Tolani
authors.affiliation: Department of Radiology, Division of Pediatric Neuroradiology, Lucile Packard Children's Hospital at Stanford, Palo Alto, California.
authors.forename: Kristen W
authors.initials: KW
authors.lastname: Yeom
authors.affiliation: Department of Neurology, Division of Child Neurology, Lucile Packard Children's Hospital at Stanford, Palo Alto, California. Electronic address: jelbers@stanford.edu.
authors.forename: Jorina
authors.initials: J
authors.lastname: Elbers
mesh_terms: D000293:Adolescent; D020771:Anterior Cerebral Artery / Q000473:pathology*; D002545:Brain Ischemia / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D002340:Carotid Artery Diseases / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D002343:Carotid Artery, Internal / Q000473:pathology*; D002533:Cerebral Angiography; D002539:Cerebral Arterial Diseases / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D020768:Middle Cerebral Artery / Q000473:pathology*; D012189:Retrospective Studies; D020521:Stroke / Q000150:complications / Q000175:diagnosis / Q000473:pathology
keywords: carotid artery dissection; focal cerebral arteriopathy; moyamoya disease; pediatric stroke