title: Vascular Imaging Outcomes of Childhood Primary Angiitis of the Central Nervous System.
abstract: Inflammation affecting cerebral blood vessels is a common cause of stroke in children. Arterial abnormalities on vascular imaging are an important risk factor for stroke recurrence. We aimed to describe the vascular imaging outcomes in children with primary angiitis of the central nervous system after 12 months and identify factors associated with vascular progression and stroke recurrence.
  
  
  
  We retrospectively analyzed clinical and neuroimaging data from the BrainWorks Registry of children with large-vessel primary angiitis of the central nervous system. Neuroimaging was collected at baseline and at least 12-month follow-up, and vascular outcome was categorized as improved, stable, or progressed based on comparison of magnetic resonance angiography. Univariate clinical and neuroimaging predictors were associated with outcome by Fisher exact test.
  
  
  
  Our study consisted of 27 children; 20 male; median age was 7.92 years (range, two to 15 years). Twelve patients received steroids (44%). Median follow-up time was 16 months (range, 12 to 56 months). Vascular imaging outcome was categorized as improved in 37%, stable in 22%, and progressed in 41% of patients. Discordant progression, defined as progression and improvement occurring simultaneously across multiple vessels, was observed in 26%. Stroke recurred in 15%, occurring exclusively in the group with progression on follow-up imaging (P = 0.02).
  
  
  
  After 12 months, 40% of children with primary angiitis of the central nervous system demonstrated progression on vascular imaging, without apparent clinical or angiographic predictors. Stroke recurrence was associated with vascular progression. Discordant progression is a newly described angiographic finding. Further studies are necessary to determine if this represents a unique characteristic of inflammatory arteriopathies.
authors.affiliation: Division of Child Neurology, Department of Neurology and Neurological Sciences, Stanford University, Stanford, California. Electronic address: jelbers@stanford.edu.
authors.forename: Jorina
authors.initials: J
authors.lastname: Elbers
authors.affiliation: Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Derek
authors.initials: D
authors.lastname: Armstrong
authors.affiliation: Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Ivanna
authors.initials: I
authors.lastname: Yau
authors.affiliation: Division of Rheumatology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Susanne
authors.initials: S
authors.lastname: Benseler
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000000981:diagnostic imaging* / Q000503:physiopathology
keywords: angiography; central nervous system; child; inflammation; outcome; vasculitis