Background: Cerebral arteriopathy is a risk factor for incident and recurrent childhood AIS. There are no standardized criteria to quantify arteriopathy severity.
Aims: 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.
Keywords: cerebrovascular disease; childhood stroke; magnetic resonance imaging; method; risk factors; stenosis.
© 2014 World Stroke Organization.