Spectrum of cerebral arteriopathies in children with arterial ischaemic stroke

Objective: To determine that children with arterial ischaemic stroke due to an identifiable arteriopathy are distinct from those without arteriopathy, and that each arteriopathy subtype has unique and recognizable clinical features.

Methods: We report a large, observational, multicenter cohort of children with AIS, age 1-month to 18-years, enrolled into the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared using the Fisher exact test, with linear step-up permutation min-p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between AIS cases with and without arteriopathy and between arteriopathy subtypes.

Results: Of 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included: dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy–inflammatory subtype (FCA-i, 15%), diffuse cerebral vasculitis (15%), and non-specific arteriopa...