Predictive validity of severity grading for cerebral steno-occlusive arteriopathy in recurrent childhood ischemic stroke.

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.

Review of lipid and lipoprotein(a) abnormalities in childhood arterial ischemic stroke.

National organizations recommend cholesterol screening in children to prevent vascular disease in adulthood. There are currently no recommendations for cholesterol and lipoprotein (a) testing in children who experience an arterial ischemic stroke. While dyslipidemia and elevated lipoprotein (a) are associated with ischemic stroke in adults, the role of atherosclerotic risk factors in childhood arterial ischemic stroke is not known. A review of the literature was performed from 1966 to April 2012 to evaluate the association between childhood arterial ischemic stroke and dyslipidemia or elevated lipoprotein (a). Of 239 citations, there were 16 original observational studies in children (with or without neonates) with imaging-confirmed arterial ischemic stroke and data on cholesterol or lipoprotein (a) values. Three pairs of studies reported overlapping subjects, and two were eliminated. Among 14 studies, there were data on cholesterol in 7 and lipoprotein (a) in 10. After stroke, testing was performed at >three-months in nine studies, at ≤three-months in four studies, and not specified in one study. There were five case-control studies: four compared elevated lipoprotein (a) and one compared abnormal cholesterol in children with arterial ischemic stroke to controls. A consistent positive association between elevated lipoprotein (a) and stroke was found [Mantel-Haenszel OR 4·24 (2·94-6·11)]. There was no association in one study on total cholesterol, and a positive association in one study on triglycerides. The literature suggests that elevated lipoprotein (a) may be more likely in children with arterial ischemic stroke than in control children. The absence of confirmatory study on dyslipidemia should be addressed with future research.