Inflammatory Type Focal Cerebral Arteriopathy of the Posterior Circulation in Children: A Comparative Cohort Study

Author:

Slavova Nedelina123ORCID,Muenger Robin4ORCID,Sanchez-Albisua Iciar4,Regényi Maria4,Oesch Gabriela4,Fluss Joël5ORCID,Hackenberg Annette6,Lebon Sébastien7ORCID,Maier Oliver8,Datta Alexandre N.9,Bigi Sandra1011ORCID,Grunt Sebastian4,Steinlin Maja4ORCID

Affiliation:

1. Support Center for Advanced Neuroimaging, Institute of Diagnostic and Interventional Neuroradiology (N.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.

2. Pediatric Radiology, University of Basel Children’s Hospital and University of Basel, Switzerland (N.S.).

3. Department of Neurology, University Hospital Bern (N.S.), University of Bern, Switzerland.

4. Division of Neuropaediatrics, Development and Rehabilitation, Department of Paediatrics (R.M., I.S.-A., M.R., G.O., S.G., M.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.

5. Department of Pediatrics, Gynecology and Obstetrics, Pediatric Neurology Unit, University Hospitals of Geneva, Switzerland (J.F.).

6. Department of Pediatric Neurology, University Children’s Hospital, Zürich, Switzerland (A.H.).

7. Pediatric Neurology and Neurorehabilitation Unit, Lausanne University Hospital, Switzerland (S.L.).

8. Division of Child Neurology, Department of Pediatrics, Children’s Hospital, St. Gallen, Switzerland (O.M.).

9. Department of Pediatric Neurology and Developmental Medicine, University of Basel Children’s Hospital, Switzerland (A.D.).

10. Institute of Social and Preventive Medicine (S.B.), University of Bern, Switzerland.

11. Division of Pediatric Neurology, Department of Pediatrics, Children’s Hospital Lucerne, Switzerland (S.B.).

Abstract

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

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