title: Predictive validity of severity grading for cerebral steno-occlusive arteriopathy in recurrent childhood ischemic stroke.
abstract: BACKGROUND
  Cerebral arteriopathy is a risk factor for incident and recurrent childhood AIS. There are no standardized criteria to quantify arteriopathy severity.
  
  
  OBJECTIVE
  To evaluate a method of scoring severity of steno-occlusive arteriopathy in childhood arterial ischemic stroke (AIS) and its association with recurrence.
  
  
  METHODS
  In a single-center prospectively enrolled cohort of 49 children with first AIS and arteriopathy, a composite cerebrovascular stenosis score (CVSS) was measured by two independent raters as the sum of stenosis scores in each of 18 intracranial large and medium arteries, where 0 = none; 1 = low-grade, 1-50%; 2 = high-grade, >50-99%; 3 = occlusion, 100%. Cox proportional-hazards models were used to determine the association of CVSS with recurrence. The analysis was stratified by presence or absence of moyamoya arteriopathy (syndrome or disease).
  
  
  RESULTS
  At a median follow-up period of 2.5 years (range: 0.8-9), 18/49 children (37%) experienced a recurrence. Median time to recurrence was 0.2 (range: 0.02-2.8) years. Interrater agreement was good, with an intraclass correlation coefficient of 0.77 [95% confidence interval (CI) 0.63-0.87, P < 0.001). Higher CVSS was associated with higher recurrence rate [hazard ratio (HR) per point 1.09, 95% CI 1.04-1.16, P = 0.001]. Among those with moyamoya arteriopathy, CVSS was associated with recurrence (HR per point of CVSS 1.11, 95% CI 1.03-1.19, P = 0.004), but there was no association in those without moyamoya arteriopathy (HR per point of CVSS 0.91, 95% CI 0.75-1.09, P = 0.32).
  
  
  CONCLUSIONS
  The CVSS is a reliable measure of severity of steno-occlusive arteriopathy in childhood stroke. This preliminary study suggests that higher CVSS is associated with stroke recurrence in children with moyamoya arteriopathy.
authors.affiliation: Department of Neurology, Neurological Institute of New York, Columbia University Medical Center, New York, NY, USA.
authors.forename: Sally M
authors.initials: SM
authors.lastname: Sultan
authors.forename: Lauren A
authors.initials: LA
authors.lastname: Beslow
authors.forename: Arastoo
authors.initials: A
authors.lastname: Vossough
authors.forename: Mitchell S V
authors.initials: MS
authors.lastname: Elkind
authors.forename: Scott E
authors.initials: SE
authors.lastname: Kasner
authors.forename: David M
authors.initials: DM
authors.lastname: Mirsky
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Licht
authors.forename: Rebecca N
authors.initials: RN
authors.lastname: Ichord
mesh_terms: D000293:Adolescent; D002545:Brain Ischemia / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D002539:Cerebral Arterial Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012008:Recurrence; D012307:Risk Factors; D012720:Severity of Illness Index; D020521:Stroke / Q000150:complications / Q000175:diagnosis* / Q000473:pathology
keywords: cerebrovascular disease; childhood stroke; magnetic resonance imaging; method; risk factors; stenosis
references.citation: J Child Neurol. 2000 May;15(5):290-4
references.pmid: 10830194
references.citation: Ann Neurol. 2011 Jan;69(1):130-40
references.pmid: 21280083
references.citation: Lancet. 2003 Jan 11;361(9352):107-16
references.pmid: 12531577
references.citation: Neurology. 1995 Aug;45(8):1488-93
references.pmid: 7644046
references.citation: AJNR Am J Neuroradiol. 2000 Mar;21(3):549-56
references.pmid: 10730650
references.citation: Pediatrics. 2007 Mar;119(3):495-501
references.pmid: 17332202
references.citation: Stroke. 2012 Apr;43(4):967-73
references.pmid: 22308239
references.citation: Brain Dev. 2008 Jan;30(1):14-9
references.pmid: 17573220
references.citation: Circulation. 2006 Nov 14;114(20):2170-7
references.pmid: 17075014
references.citation: Curr Opin Pediatr. 2004 Dec;16(6):617-22
references.pmid: 15548922
references.citation: Ann Neurol. 2006 Apr;59(4):620-6
references.pmid: 16450385
references.citation: Biometrics. 1977 Mar;33(1):159-74
references.pmid: 843571
references.citation: Acta Paediatr. 2009 Oct;98(10):1613-9
references.pmid: 19594463
references.citation: Neurology. 2007 Jun 12;68(24):2099-106
references.pmid: 17409371
references.citation: Cerebrovasc Dis. 2005;20(5):347-54
references.pmid: 16131804
references.citation: Arch Neurol. 1969 Mar;20(3):288-99
references.pmid: 5775283
references.citation: Neurosurg Focus. 2011 Dec;31(6):E6
references.pmid: 22133171
references.citation: Brain. 2009 Feb;132(Pt 2):544-57
references.pmid: 19039009
references.citation: J Child Neurol. 2011 Sep;26(9):1203-6
references.pmid: 21743063