Inflammatory Type Focal Cerebral Arteriopathy of the Posterior Circulation in Children: a comparative cohort study

Abstract

AbstractBackgroundInflammatory type Focal Cerebral Arteriopathy (FCA-i) in the anterior circulation (AC) is well characterized and the FCA severity score (FCASS) reflects the severity of disease. We identified cases of FCA-i in the posterior circulation (PC) and adapted the FCASS to describe these cases.MethodsPatients from the Swiss NeuroPaediatric Stroke Registry (SNPSR) with ischemic stroke in the PC and AC due to FCA-i and available neuroimaging were gathered. Comparison of data regarding Pediatric National Institutes of Health Stroke Scale (pedNIHSS) score and Pediatric Stroke Outcome Measure (PSOM) and FCASS was performed. We estimated infarct size by the modified pediatric Alberta Stroke Program Early Computed Tomography Score (pedASPECTS) in children with AC stroke and the adapted Bernese posterior diffusion-weighted imaging (DWI) score in the PC.ResultsThirty-six children with a median age of 6.3 years ([IQR 2.8,8.6; range 0.9,15.6], 21 males, 58.3%) with FCA-i were identified. The total incidence rate was 0.151/100 000/year (95%CI 0.109–0.209). Seven had PC FCA-i and 5 had FCA-i in both circulations. Time to final FCASS was longer in the PC compared to AC, evolution of FCASS did not differ. Initial pedNIHSS was highest in children with FCA-i in the PC with a median of 8.0 (IQR 5.0-18.0), compared to 4.5 (IQR 2.0-8.0) in those with AC FCA-i and 6.0 (IQR 6.0-6.0) with involvement of both AC and PC. Different to the anterior cases PC infarct volume did not correlate with higher discharge, maximum or final FCASS scores (R 0.25, 0.35, 0.54).ConclusionThe PC is affected in up to one third of cases of FCA-i. These cases should be included in future investigations on FCA-i. Although it did not correlate with clinical outcome in our cohort, the modified FCASS may well serve as a marker for the evolution of the arteriopathy in posterior FCA-i.