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Published on: February 5, 2011
Predictive validity of severity grading for cerebral steno-occlusive arteriopathy in recurrent childhood ischemic
Sally M Sultan1, Lauren A Beslow, Arastoo Vossough
1Department of Neurology, Neurological Institute of New York, Columbia University Medical Center, New York, NY, USA.
Insights
A new scoring system, the composite cerebrovascular stenosis score (CVSS), reliably measures arteriopathy severity in children with stroke. Higher CVSS scores indicate increased risk of stroke recurrence, particularly in those with moyamoya arteriopathy.
Area of Science:
- Pediatric Neurology
- Vascular Neurology
- Medical Imaging Analysis
Background:
- Childhood arterial ischemic stroke (AIS) is often linked to cerebral arteriopathy.
- Standardized methods for quantifying arteriopathy severity in pediatric AIS are lacking.
- Arteriopathy severity is a key factor in predicting stroke recurrence.
Purpose of the Study:
- To assess the reliability of a novel scoring method, the composite cerebrovascular stenosis score (CVSS), for quantifying arteriopathy severity in pediatric AIS.
- To investigate the association between CVSS and the rate of stroke recurrence in children.
- To explore if this association differs between patients with and without moyamoya arteriopathy.
Main Methods:
- A prospective cohort of 49 children with first AIS and arteriopathy was studied.
- The CVSS was calculated by summing stenosis scores across 18 intracranial arteries (0-3 scale).
- Cox proportional-hazards models analyzed the link between CVSS and recurrence, stratified by moyamoya presence.
Main Results:
- A good interrater agreement (ICC=0.77) was found for the CVSS.
- Higher CVSS scores were significantly associated with an increased rate of stroke recurrence (HR=1.09 per point).
- This association was significant in children with moyamoya arteriopathy (HR=1.11 per point) but not in those without.
Conclusions:
- The CVSS demonstrates reliability for assessing steno-occlusive arteriopathy severity in pediatric stroke.
- Elevated CVSS scores may predict stroke recurrence in children, especially those with moyamoya arteriopathy.
- Further research is warranted to validate CVSS in larger, diverse pediatric populations.
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.
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