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Inflammatory Type Focal Cerebral Arteriopathy of the Posterior Circulation in Children: A Comparative Cohort Study
Nedelina Slavova1,2,3, Robin Muenger4, Iciar Sanchez-Albisua4
1Support Center for Advanced Neuroimaging, Institute of Diagnostic and Interventional Neuroradiology (N.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.
Insights
Inflammatory focal cerebral arteriopathy (FCA-i) affects the posterior circulation (PC) in children. Adapted severity scores may track disease evolution in PC-FCA-i cases.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Cerebrovascular Diseases
Background:
- Focal cerebral arteriopathy, inflammatory type (FCA-i) in the anterior circulation (AC) is well-defined.
- The Focal Cerebral Arteriopathy Severity Score (FCASS) quantifies disease severity in AC cases.
- This study investigates FCA-i in the posterior circulation (PC) and adapts FCASS for PC cases.
Purpose of the Study:
- To identify and characterize pediatric cases of FCA-i in the posterior circulation (PC).
- To compare clinical and imaging features of PC-FCA-i with anterior circulation (AC)-FCA-i.
- To evaluate the utility of an adapted FCASS for PC-FCA-i.
Main Methods:
- Comparative cohort study of pediatric ischemic stroke patients with FCA-i (2000-2018).
- Analysis of data from the Swiss NeuroPaediatric Stroke Registry.
- Comparison of clinical severity (pediatric NIH Stroke Scale) and infarct size (modified pediatric Alberta Stroke Program Early Computed Tomography Score for AC, adapted Bernese posterior diffusion-weighted imaging score for PC) between PC and AC cases.
Main Results:
- Thirty-five children with FCA-i were identified; six had PC involvement.
- Children with PC-FCA-i presented with higher initial NIH Stroke Scale scores compared to AC-FCA-i.
- In PC cases, infarct volume did not correlate with FCASS scores, unlike in AC cases.
Conclusions:
- FCA-i is also present in the posterior circulation (PC) and should be included in future research.
- An adapted FCASS may serve as an evolution marker for PC-FCA-i, despite not correlating with clinical outcomes in this cohort.
Background:
Inflammatory type focal cerebral arteriopathy (FCA-i) in the anterior circulation (AC) is well characterized, and the focal cerebral arteriopathy severity score (FCASS) reflects the severity of the disease. We identified cases of FCA-i in the posterior circulation (PC) and adapted the FCASS to describe these cases.
Methods:
In this comparative cohort study, patients from the Swiss NeuroPaediatric Stroke Registry with ischemic stroke due to FCA-i between January 2000 and December 2018 were analyzed. A comparison between PC and AC cases regarding pediatric National Institutes of Health Stroke Scale score and pediatric stroke outcome measure and FCASS was performed. We estimated infarct size by the modified pediatric Alberta Stroke Program Early Computed Tomography Score in children with AC stroke and the adapted Bernese posterior diffusion-weighted imaging score in the PC.
Results:
Thirty-five children with a median age of 6.3 (interquartile range, 2.7-8.2 [95% CI, 0.9-15.6]; 20 male; 57.1%) years with FCA-i were identified. The total incidence rate was 0.15/100 000/year (95% CI, 0.11-0.21). Six had PC-FCA-i. Time to final FCASS was longer in the PC compared with AC; the evolution of FCASS did not differ. Initial pediatric National Institutes of Health Stroke Scale score was higher in children with FCA-i in the PC with a median of 10.0 (interquartile range, 5.75-21.0) compared with 4.5 (interquartile range, 2.0-8.0) in those with AC-FCA-i. Different from the anterior cases, PC infarct volume did not correlate with higher discharge, maximum, or final FCASS scores (Pearson correlation coefficient [r], 0.25, 0.35, and 0.54).
Conclusions:
FCA-i also affects the PC. These cases should be included in future investigations into FCA-i. Although it did not correlate with clinical outcomes in our cohort, the modified FCASS may well serve as a marker for the evolution of the arteriopathy in posterior FCA-i.
